Hematology Q&A USMLE

27-yo white woman presents to GYN because of pelvic pain. She has experienced painful periods since beginning menses at age 12 years. The pain is sometimes present when she is not menstruating, and has become progressively worse over the years. She experienced some improvement during the 4 years she took oral contraceptives, which she stopped taking 2 years ago when she got married. She and her husband have been trying to conceive for the past 18 months without success. Her current menstrual cycle is 25-30 days, with heavy periods lasting 5-7 days. Her last menstrual period was 3 weeks ago and was accompanied by severe cramps that were unresponsive to ibuprofen and prevented her from going to work for 2 days. The pain usually improves with onset of menses, and is aggravated by pain with intercourse and bowel movements. Her β-human chorionic gonadotropin test is negative. Which of the following is the best test for diagnosing her underlying condition? (A) Diagnostic laparoscopy with biopsies (B) Endometrial biopsy (C) Hysterosalpingography (D) Measurement of prostaglandin F2α level (E) Pelvic ultrasound

A. (Endometriosis is a condition in which endometrial tissue abnormally implants at sites outside the uterus. Ovaries are the most common site of involvement, but implantation in extrapelvic sites such as the umbilicus, lungs (pretty rare, but documented), and surgical scars has also been documented. Most cases occur in women 20-30 years old, and it is rarely seen after menopause. Endometriosis may be asymptomatic, but it may also cause menorrhagia, severe pelvic pain, and infertility, as seen in this patient. The amount of pain is often out of proportion to the number of extrauterine implants. Like endometrial tissue in the uterine cavity, extrauterine endometrial tissue is affected by hormonal cycles and symptoms may improve with use of oral contraceptive pills. Pathologic review of biopsied lesions is required for diagnosis, and the gold standard for doing so is laparoscopy)

A 64-year-old woman with a history of cardiac disease, multiple strokes, and progressive osteoarthritis is admitted to the hospital for bilateral total knee replacement surgery. The morning after surgery her platelet count is 9000/ mm³. Which of the following medications is most likely responsible for this finding? (A) Aspirin (B) Clopidogrel (C) Heparin (D) Streptokinase (E) Warfarin

C. (HIT is a well-established adverse effect of treatment and may occur in as many as 20% of patients receiving heparin therapy. Thrombocytopenia most commonly occurs after 4-10 days of treatment. The more severe form of HIT, type II HIT, is caused by antibodies against the heparin-platelet factor 4 complex. Patients on chronic warfarin therapy (e.g., those with a history of cardiac disease and multiple strokes) are transitioned to the shorter-acting heparin prior to major surgery, thus explaining the time course of this patient's reaction)

It is a common cause of secondary dilated cardiomyopathy. Echocardiography demonstrates increased left ventricular size and diminished ejection fraction.

Ischemic cardiomyopathy

An intensive multiagent chemotherapeutic regimen that is often used to treat intermediate- and highgrade lymphomas. It would not be indicated in treatment of low-grade disease because it has a severe adverse effect profile with low success in indolent disease.

The CHOP protocol (Cyclophosphamide, Hydroxydaunomycin, Oncovorin [vincristine], and Prednisone)

Name 3 Causes of Restrictive Cardiomyopathy

amyloidosis, hemochromatosis, and sarcoidosis

4-yo boy presents with increased fatigue past 4 months. He also has intermittent fevers that resolve on their own and progressively more frequent nosebleeds, up to 3 times a week. His mother notes that he has generally stopped walking, but when he tries to walk he seems to favor his left leg. PE shows* pallor, cervical lymphadenopathy, hepatomegaly, splenomegaly, and crusted blood on the nares*. His WBC count is 28,000/mm³, with a hemoglobin level of 8.3 g/dL and a decreased platelet count. Bone marrow biopsy shows increased cellularity and lymphoblast predominance. Which of the following is true of this patient's likely diagnosis? (A) Children with trisomy 21 are at increased risk of developing this disease (B) Presence of the Philadelphia chromosome means that there is no current treatment (C) Survival with chemotherapy is approximately 20%-30% in children (D) The central nervous system is usually spared from metastatic spread (E) Tumor lysis syndrome is uncommon

A. (ALL is a disease of hyperproliferative immature lymphocytes and occurs mainly in children, with a second peak in persons who are elderly. It is associated with a number of other diseases, including Down's syndrome, Fanconi's anemia, and ataxia telangiectasia)

49-yom with history of several dysplastic nevi presents for an annual examination. He mentions that one of the moles on his left shoulder seems larger than usual. It has also started to itch. He denies anorexia, weight changes, or malaise, though he admits to feeling somewhat tired in the past couple of months. On exam he is well appearing and has no lymphadenopathy. The mole is dark brown, 7 mm in diameter, nodular, and round with an irregular shape. There is no ulceration or bleeding. Which of the following is the most appropriate next step in management? (A) Excision of the lesion (B) Excision of the lesion with sentinel lymph node biopsy (C) Local radiation therapy (D) Oral antihistamines for symptomatic relief (E) Reassurance, with follow-up in 6 months

A. (Any description of a changing mole is concerning for occurrence of melanoma. The ABCDE mnemonic is often used to remember the key changes that indicate development of malignancy (Asymmetry, irregular Borders, Color changes or irregularity, Diameter increasing, and Evolution or changing of the mole). Several features of this mole are particularly concerning, including the size (>6 mm) and presence of itching. The treatment of choice is excisional biopsy, with margins determined by apparent depth of the lesion)

65-yo, otherwise healthy woman presents with shortness of breath. PE reveals JVD and bilateral lower extremity edema. Echocardiography shows enlarged left ventricular end-systolic and end-diastolic dimensions, an ejection fraction of 45%, and increased left ventricular wall thickness. Which of the following is the most likely cause? (A) Amyloidosis (B) Chagas' disease (C) Doxorubicin (D) Idiopathic hypertrophic subaortic stenosis (E) Ischemic cardiomyopathy

A. (The patient presents with symptoms of right-sided heart failure. Echocardiographic results are consistent with a restrictive cardiomyopathy, which leads to impaired elasticity. Causes of restrictive cardiomyopathy include amyloidosis, hemochromatosis, and sarcoidosis)

22-yo man who has recently moved to USA from eastern Africa presents to ER department with severe abdominal pain. He states the pain began 30-40 min earlier and pain is currently 10 of 10. He denies vomiting or diarrhea. While in the ER, he passes a bloody stool. Examination demonstrates an acute abdomen and the patient is taken to the operating room. On laparotomy an intussusception caused by tremendous swelling of the intestinal wall is discovered. A large section of intestine is resected. Pathologic examination of frozen sections reveals lymphoid tissue. Which of the following is the most likely diagnosis? (A) Burkitt's lymphoma (B) Chronic lymphocytic leukemia (C) Chronic myelogenous leukemia (D) Ewing's sarcoma (E) Sézary syndrome

A. (The presence of intussusception in an adult or older child should raise suspicion for Burkitt's lymphoma. This disease is more common in patients living in Africa, an association believed to be due to the high rate of infection with Epstein-Barr virus. The African form tends to involve the maxilla and mandible rather than the abdomen. However, the African form of Burkitt's lymphoma can affect the Peyer's patches of the intestine, causing tremendous swelling that leads to foci for intussusception. The 8;14 translocation, which is associated with the c-myc oncogene, is commonly found in Burkitt's lymphoma. This disease is a high-grade lymphoma that is very responsive to chemotherapy)

63-yo postmenopausal woman is referred to the GY clinic by her primary care for evaluation of genital pruritus of 1 months' duration. She has a remote history of human papillomavirus infection. She denies changes in vaginal discharge or vaginal bleeding. She also denies constitutional symptoms or weight loss. On PE she is in no apparent distress and HR is 70/min, BP is 100/58, and RR is 10/min. Genital exam reveals an ulcerative white lesion approximately 1 cm in diameter on her labia majora. What is the most appropriate next step in management? (A) Obtain a biopsy of the lesion (B) Prescribe estrogen cream to be applied to area (C) Treat with acyclovir (D) Treat with cryotherapy (E) Treat with fluconazole (F) Treat with metronidazole

A. (This is a common presentation of vulvar carcinoma. Typically it presents in women 65-70 years of age, and the most common presenting complaint is vulvar pruritus. Previous HPV infection is a major risk factor for the development of vulvar carcinoma. Definitive diagnosis requires a biopsy. Spread is by local invasion and then to regional lymph nodes, so treatment includes wide surgical excision and regional lymph node excision.)

7-yo boy presents to his pediatrician because of increased gingival bleeding after brushing his teeth. The patient's mother denies a history of easy bruising or prolonged bleeding. The boy also reports an episode of prolonged and painful knee swelling after a fall in which he hit his knee. The patient's family history is significant for a maternal grandfather who died of a massive hemorrhage after a minor surgical procedure. On PE there are no ecchymoses or petechiae. His conjunctivae are pink and a full physical examination is noncontributory. His activated partial thromboplastin time is 63 sec, prothrombin time is 12 sec, bleeding time is 4 min, and coagulation time is prolonged. Which of the following additional laboratory results is most likely to be seen in this patient? (A) Decreased factor VIII concentrations (B) Decreased platelet concentrations (C) Decreased WBC count (D) Increased factor V concentrations (E) Increased hemoglobin

A. (This patient has hemophilia. This diagnosis is based on a history of mucosal bleeding and hemarthrosis along with a family history of a bleeding disorder that is consistent with an X-linked recessive inheritance pattern (the patient's mother is a carrier of the disease). Patients with hemophilia do not present with ecchymoses or petechiae as may be seen in other bleeding disorders such as vWD. Hemophilia presents with an increased activated partial thromboplastin time because of deficiencies in components of the intrinsic pathway. The prothrombin time is normal in these patients because their extrinsic coagulation pathway remains intact. Finally, bleeding time is normal in these patients because neither their platelet concentration nor function is altered)

72-yo man with 40-pack-year smoking history presents with a 9-kg (20-lb) weight loss and fatigue. He has no other complaints. He is not taking any medication. PE and vital signs are unremarkable. Laboratory tests show: Na+: 138; K+: 4.6; Cl−: 101;HCO3−: 24 mEq/L Ca2+: 11.2 mg/dL; PO4: 1.6 mg/dL; Mg2+: 2.0 mg/dL BUN: 11 mg/dL; Cr: 1.1 mg/dL; Glucose: 94 mg/dL Parathyroid hormone: 12 pg/mL Which of the following is the best next step in the evaluation of this patient? (A) CT of the chest (B) MRI of the brain (C) Parathyroid ultrasound (D) Renal biopsy (E) Serum vitamin D levels

A. (This patient has hypercalcemia and hypophosphatemia. This is consistent with hyperparathyroidism; however, the PTH level is low to normal. In addition to parathyroid adenomas, malignancy can cause hypercalcemia by the release of PTHrP. PTHrP mimics the action of PTH, and causes hypercalcemia by increased bone resorption and increased distal tubular reabsorption of calcium in the kidney. It causes hypophosphatemia by inhibiting proximal tubular reabsorption of phosphate. Malignancy (particularly lung cancer, breast cancer, and multiple myeloma) should be suspected in patients with unexplained hypercalcemia and low or normal PTH. This is especiallytrue in this patient who has a significant smoking history and weight loss)

67-yo man presents with flu-like symptoms and cough. He also reports 5-lb weight loss over past 2 months. He has 40-pack-year history of smoking but quit 10 years ago. His past medical history and PE are unremarkable. CBC and liver function tests are normal. His stool is negative for occult blood. X-ray of the chest revealed a 3.5-cm right lobe mass and widening of the mediastinum. CT scan shows a right middle lobe mass, and in addition two mediastinal lymph nodes are seen that measure 1.5 cm and 2 cm in diameter, respectively. Biopsies of the lung nodule obtained by bronchoscopy indicate malignancy. Which of the following is the best next best step in management? (A) Mediastinoscopy (B) Pulmonary function tests (C) Thoracentesis (D) Ventilation-perfusion lung scan (E) Video-assisted thoracoscopic surgery

A. (This patient is symptomatic, a smoker, and has a right lobe mass and mediastinal lymph node involvement that require immediate evaluation. Nodes <2 cm in diameter are typically benign nodes, whereas nodes >2 cm are more likely to be malignant. The status of his nodes would provide critical information necessary for this patient's management. Therefore, the next appropriate step would be to evaluate the nodes by mediastinoscopy. If the nodes are benign, the patient would be a good candidate for surgical resection. However, if the nodes are malignant, other modalities of treatment would be indicated)

51-yo woman undergoes a successful *bone marrow transplant* from a matched unrelated donor for refractory Hodgkin's disease. She is discharged from the hospital on no medications and is feeling well. At an appointment 6 months post-transplant she is well with no evidence of malignancy. 3 weeks later she travels to Florida with her family. She is cautious of the sun but develops sunburn on her face, despite wearing sunscreen and a protective hat. When she returns from her trip 5 days later she presents with persistent erythema of her face. She also states her *wrists and hands have been sore for the past 2-3 weeks*. On PE her face is mildly tender to touch, and a rash is present. Her hands are diffusely swollen. She is afebrile, and the remainder of her PE is benign. What is the most likely diagnosis? (A) Graft-versus-host disease (B) Hypersensitivity to sunlight due to the patient's antirejection regimen (C) New-onset systemic lupus erythematosus (D) Rosacea (E) Staphylococcal skin infection acquired during travel (F) Sun poisoning

A. (Twenty to 50% of patients with a history of allogeneic bone marrow transplant develop GVHD. GVHD is caused by a proliferation of grafted donor T lymphocytes that ultimately reject the host's foreign proteins, leading to end-organ damage. This disease is a separate entity from acute GVHD and resembles an autoimmune disorder. It may result in macular-papular rash, as seen in this patient; sicca syndrome; arthritis; and bile duct degeneration, leading to jaundice. The chronic form of GVHD may occur months or even years after transplant and is likely due to vascular damage by the proliferated T lymphocytes. Patients who are >40 years at the time of transplant are at the greatest risk of developing chronic GVHD. This complication is irreversible, but prednisone is the standard of care)

positive myeloperoxidase test is diagnostic for ____

AML

Affects children much more often than adults. It may result in pancytopenia. Immature blasts are evident on the peripheral smear of patients

Acute lymphocytic leukemia (ALL)

29-yo woman presents to an obstetrics clinic asking for a pregnancy test. Her LMP was 7 weeks ago. She took home pregnancy test and it was positive. She says that this is a highly desired pregnancy, and that she and her partner have been attempting to become pregnant for almost a year. She says that she has no other complaints, and says that she "has never felt better." Her pulse is 78/min, BP is 110/70 mm Hg, and she is afebrile. Pelvic examination is within normal limits. The physician notices, however, an abnormality on breast exam, which reveals a 3-cm mass palpated just above the nipple.The mass is mobile and well circumscribed. The patient says that she noticed it before, and that it has gotten larger recently. However, she didn't think it was important because it didn't hurt. Which of the following is the most likely diagnosis? (A) Carcinoma of the breast (B) Fibroadenoma (C) Fibrocystic change (D) Galactocele (E) Mammary duct ectasia

B. (A fibroadenoma is a benign and slow-growing breast tumor with epithelial and stromal components. It is the most common lesion in women <30 years old. This patient's presentation of a sharply demarcated, mobile, nontender mass is classic for fibroadenoma. In addition, these lesions often get larger with pregnancy. These tumors are usually removed when they reach 2-4 cm in diameter)

28-yo woman is diagnosed with acute promyelocytic leukemia (APL) after experiencing severe fatigue and malaise for several weeks. She and her parents have done extensive reading on the Internet and expect that she will be treated with intensive multiagent chemotherapy. To their surprise, her physician suggests an oral agent that is not used to treat other forms of acute leukemia. Why might the physician be concerned about using standard intensive multiagent chemotherapy in this patient? (A) A desire to allow the patient to preserve her fertility (B) A higher incidence of chemotherapy related death in patients with APL as compared to other leukemias (C) Personal preference to treat APL with homeopathic agents (D) An observation that APL does not respond to conventional chemotherapeutics (E) An understanding that APL is so sensitive to oral agents that intravenous chemotherapy is simply not required in its treatment

B. (Although APL is generally responsive to the chemotherapeutics used to treat acute myelogenous leukemia (AML), cytarabine and daunorubicin, approx 10% of APL patients treated on this regimen die from disseminated intravascular coagulation. This phenomenon occurs when dying leukemic promyelocytes release their granule components. Modern protocols use tretinoin, an oral drug that promotes maturation of leukemic cells bearing the t(15;17) abnormality, the best known genetic marker for APL. Tretinoin is often used in combination with anthracycline chemotherapy, with a period during which tretinoin is used alone)

32-yo woman presents to ER department with *edema and pain of right lower extremity* that began after a 6-hour car ride. A Doppler ultrasound was completed in which a DVT was noted. The patient has no prior history of DVT or pulmonary emboli. The patient has been taking oral contraceptive pills for the past 2 years and is currently compliant with her medication. Her family history is significant for a maternal grandmother, mother, and sister with recurrent DVT. Her temperature is 36.2°C, BP is 112/78, HR is 86/min, RR 14/min. There is no clinical evidence indicating pulmonary embolism. Which of the following is the most likely cause of her DVT? (A) Antithrombin deficiency (B) Coagulation factor V gene mutation (C) Protein C excess (D) Protein S deficiency (E) Prothrombin gene mutation

B. (Although DVTs in this population are often attributed to the use of oral contraceptives, there is a strong family history of recurrent DVTs and therefore we must consider hereditary disorders of the coagulation system. Factor V Leiden is an autosomal dominant disorder in which coagulation factor Va (the activated form of coagulation factor V) is resistant to degradation by activated protein C. Factor Va remains active and allows for the conversion of prothrombin to thrombin, thereby promoting a hypercoagulable state in patients. Factor V Leiden accounts for 40%-50% of venous thromboemboli in cases in whom a genetic predisposition is found. A patient with a heterozygous mutation of the gene encoding factor V carries a relative risk of 7 for DVTs, while a patient with the mutation in conjunction with oral contraceptive use carries a relative risk of 35 for DVTs.)

68-yo man is treated for *non-Hodgkin's lymphoma* for past 3 weeks, and now complains of weakness and fatigue for the past day. His T is 38.5°C (101.3°F), BP is 140/88 mm Hg, and HR is 78/min. The remainder of PE is normal. Laboratory tests show: Na+: 136 mEq/L; K+: 5.8 mEq/L; Cl−: 99 mEq/L; Ca2+: 7.9 mEq/L; HCO3 −: 25 mEq/L; BUN: 9 mg/dL; Cr: 0.9 mg/dL Urate: 9.1 mg/dL. What is the most likely cause of this patient's hyperkalemia? (A) Increased gastrointestinal absorption (B) Lysis of neoplastic cells (C) Metabolic acidosis (D) Pseudohyperkalemia (E) Renal failure

B. (Although symptoms of hyperkalemia are rare and when present are nonspecific, they include lethargy and weakness. In the context of normal kidney function as well as hyperuricemia and hypocalcemia, this hyperkalemia is likely the result of the chemotherapeutic effect on his tumor. This phenomenon, known as tumor lysis syndrome, presents shortly after commencement of chemotherapy with elevations in serum urate levels, and can progress to urate-induced kidney)

59-yo man presents with 15-lb weight loss over 2 months, with occasional night sweats. He notices some abdominal fullness but no pain, and his last colonoscopy was negative for polyps. He has never smoked tobacco, but he has had prior radiation exposure as an x-ray technician. PE is significant for *splenomegaly*, with no signs of jaundice. Fecal occult blood is negative. X-ray of the chest is clear. His WBC count is 126,000/mm³, and a peripheral smear shows a leukocytosis with all stages of maturation seen and 3% blasts. Which of the following tests would confirm the diagnosis? (A) Bone marrow biopsy with hypercellularity and elevated proportion of blasts (B) Cytogenetic identification of t(9;22) (C) Elevated α-fetoprotein level (D) Lymph node biopsy showing ReedSternberg cells (E) Repeat colonoscopy showing colonic polyps

B. (CML, in its chronic phase, presents with constitutional symptoms along with abdominal fullness due to splenomegaly. Diagnosis is most common in 4h-6th decades of life, and irradiation increases risk of developing CML. Because it is a proliferation of mature myeloid cells, peripheral smear should show predominant leukocytosis with cells in all stages of maturation. Definitive diagnosis relies on identification of the Philadelphia chromosome or the bcr-abl fusion product. However, about 5% of patients have atypical CML without the presence of bcr-abl and show a poor response to therapy)

30-yo woman presents for a new patient visit. She reports *recurrent episodes of pneumonia, bronchitis, and otitis over the past 4 years*. Although her vaccinations were up to date, she developed *tetanus* following a foot laceration last year. Her lymph nodes and tonsils are enlarged. Laboratory testing reveals low IgG, IgA, and IgM levels. After referral to hematologist, who rules out other acquired and genetic causes of her hypogammaglobulinemia, she is diagnosed with common variable immunodeficiency. This woman is at the highest risk of developing which of the following condition? (A) Cardiovascular disease (B) Lymphoma (C) Miscarriage (D) Renal disease (E) Splenic autoinfarction

B. (Common variable immunodeficiency (CVID) is a syndrome characterized by hypogammaglobulinemia in combination with phenotypically normal B lymphocytes. The disease presents suddenly in 3rd or 4th decade after recurrent infections and lack of response to vaccines. It is a diagnosis of exclusion, and its pathogenesis is not well understood. Women in their 5&6 decades of life with CVID are at a 438- fold increased risk of developing lymphoma. In addition to malignancy, patients with CVID are at increased risk of chronic lung disease, autoimmune phenomenon, and chronic diarrhea)

55-yo woman with Hx of alcoholism and chronic pancreatitis (last exacerbation 2 years ago) presents to her primary care with *weight loss, pruritus, anorexia, dark urine, jaundice, yellow sclerae, and vague abdominal pain*. Which of the following physical findings would most strongly support her most likely diagnosis? (A) Chandelier sign (B) Courvoisier's sign (C) Cullen's sign (D) Grey-Turner's sign (E) Positive Murphy's sign

B. (Courvoisier's sign is a palpable nontender gallbladder as a result of common bile duct obstruction/compression by pancreatic adenocarcinoma at the head of the pancreas. Biliary obstruction below the level of the cystic duct is unlikely to be caused by stone disease.)

28-yo woman with hx of refractory promyelocytic leukemia is admitted to the hospital to undergo a bone marrow transplant from a matched unrelated donor. In the week prior to the transplant she receives myeloablative chemotherapy. She remains stable on antibacterial prophylaxis for 9 days following her transplant. On day 10 she develops large *bullae on her palms and soles* and is diagnosed with *graft-versus-host disease*, which requires longterm therapy. She is discharged home with nursing care on trimethoprim-sulfamethoxazole, glucocorticoids, and tacrolimus. On day 45 she develops *spiking fevers and rigors*. She has been compliant with her prescribed medications. Given her history, which of the following describes the most prominent infectious risk at this time? (A) Acute bacterial infection with Staph.aureus 6 weeks following transplant (B) Fungal infection due to prolonged immunosuppression (C) Gram (-) bacteremia in 1st 6 weeks following transplant (D) Pneumocystis jiroveci after being treated for hematogenous malignancy (E) Reactivation of herpes simplex virus in posttransplant period (F) Salmonella infection, given patient history of promyelocytic leukemia

B. (Fungal infections become increasingly important in bone marrow transplant after the first 7 days posttransplantion. Patients with graft-versus-host disease (GVHD) who require long-term immunosuppression are at high risk of infection by organisms such as Candida and Aspergillus. These infections can occur even after engraftment and resolution of neutropenia (which would be expected at posttransplant day 45). Other infections that are important during this time frame are cytomegaloviral pneumonia, parasitic infection, and human herpes virus 6 infection of the skin and mucous membranes.)

47-yo man presents to his primary care complaining of *fatigue* for the past several months. He says he has had poor sleep due to *sweating at night* and has lost 4.5 kg recently, although he has not been trying to lose weight. In addition, he has been suffering from *severe headaches and blurry vision* recently. On examination he is pale and thin, with multiple *ecchymoses*. Cardiac examination is significant for a II/VI systolic flow murmur. He has an *enlarged spleen* on abdominal exam. Lab tests show: WBC count: 95,000/mm³; 15% blasts; 15% bands 47% polymorphonuclear cells; 7% basophils 10% lymphocytes; Hemoglobin: 7.2 g/dL Platelet count: 90,000/mm³ Which of following is the best next step in establishing a diagnosis? (A) Coagulation studies (B) Cytogenetic studies (C) Detection of serum leukocyte alkaline phosphatase levels (D) Iron studies (E) No further studies are necessary for establishing the diagnosis

B. (Given this patient's clinical picture and laboratory results, he likely has chronic myelogenous leukemia - CML. The differential diagnosis includes acute myelogenous leukemia and myelodysplastic syndrome. A definitive diagnosis can be made by cytogenetic studies that reveal the Philadelphia chromosome (9,22 translocation), which is pathognomonic for CML. Ninety percent of patients with CML will demonstrate this translocation, and in the 10% who do not a bone marrow biopsy will be definitive)

3-yo with sickle cell anemia is brought to ER complaining of pain in his lower arms for 3 days. On PE both arms are swollen, tender, and erythematous. He is febrile, and complains of chills. Leukocytosis is seen on blood count with prominent neutrophils. Which of the following is the most appropriate treatment? (A) Calcitonin (B) Ceftriaxone (C) Ciprofloxacin (D) Etidronate (E) Vitamin D

B. (In patients with sickle cell anemia, the primary agents of osteomyelitis are Salmonella paratyphi and Staphylococcus aureus. Osteomyelitis is a common complication of sickle cell disease, as sickle cell crises may stop blood flow to portions of bones, allowing those segments of bone to become foci for infection. Any antibiotic regimen will need to cover both organisms. A third-generation cephalosporin, such as ceftriaxone, is an acceptable choice)

12-yo boy presents with pain and swelling of his left thigh, and is diagnosed with osteosarcoma. He has no other medical problems; however, his family history reveals that his mother has been treated for bilateral breast cancer and that his sister and two uncles died of "brain tumors." He is treated with multiagent chemotherapy and recovers from his disease. Several years later, a complete CBC sent on routine follow-up reveals abnormalities consistent with myelodysplastic syndrome. Within weeks he has developed a refractory form of acute myelogenous leukemia that is rapidly fatal. Which of the following syndromes is most likely affecting this patient? (A) Ataxia telangiectasia (B) Familial Li-Fraumeni syndrome (C) Von Hippel-Lindau syndrome (D) Wiskott-Aldrich syndrome (E) Xeroderma pigmentosa

B. (Li-Fraumeni syndrome is a syndrome characterized by a mutation in one copy of the p53 tumor suppressor gene. It is inherited in an autosomal dominant fashion, and predisposes patients to sarcomas, central nervous system tumors, and carcinomas. Patients are at a 100-fold risk of acquiring treatment-related AML following treatment with cytotoxic drugs. Treatment-related AML is universally refractory to known therapies.)

60-yom presents with dysphagia, facial flushing, diarrhea. PE reveals a goiter and mild cervical lymphadenopathy. Based on thyroid function test results, he is referred to a surgeon for total thyroidectomy, which reveals medullary carcinoma. He is currently being treated with thyroid hormone suppression therapy. Which of the following is the most significant risk factor for this form of thyroid cancer? (A) Alcohol (B) Genetic risk factor (C) Iodine exposure (D) Radiation to the neck for enlarged tonsils as a child (E) Smoking (F) Viral infection

B. (Medullary carcinoma accounts for about 5% of all thyroid cancers and is familial in up to 25% of cases. MEN consists of three syndromes featuring tumors of endocrine glands, each with its own characteristic pattern. Specifically, medullary thyroid cancer is associated with MEN type IIA, as well as pheochromocytoma and parathyroid hyperplasia/tumor causing hyperparathyroidism. In MEN type IIA and familial medullary thyroid carcinoma, most patients present in the third decade of life, but screening of family members is important at an early age so patients can inform their children of the condition. Mutations in ret proto-oncogene are responsible for approximately >90% of cases of MEN type IIA. Papillary and anaplastic carcinomas have not been found to have a genetic risk factor to date.)

63-yo African-American man with no past medical history presents with a recent increase in prostate-specific antigen from 2.2 to 4.3 ng/mL. Digital rectal exam reveals 0.5 cm nodule on left lobe of prostate. Which of the following is the most appropriate next step in the evaluation of this patient? (A) CT scan and bone scan (B) Prostate biopsy (C) Radical prostatectomy (D) Treat patient with a 7-day course of antibiotics and recheck prostate-specific antigen level in 3 months (E) Urinalysis

B. (Patients with a recent elevation of prostate-specific antigen (PSA) levels and a positive digital rectal examination (DRE) are at high risk for prostate cancer. The next appropriate step is a biopsy to assess whether cancer is present. Later signs and symptoms can include nocturia, dysuria, hesitancy, sexual dysfunction, or bone pain. Once diagnosed, more tests and analyses are performed to determine the staging and grading of the disease. Treatment can include watchful waiting, surgery, radiation therapy, high-intensity focused ultrasound, chemotherapy, cryosurgery, hormonal therapy, or some combination of these.)

30-yo man presents for a routine physical. On questioning he comments that his father was diagnosed with colon cancer at 45 years of age. The patient has never had polyps and does not suffer diarrhea, constipation, or bloody stools. He is nervous about screening and wants to delay as long as possible. According to current recommendations, when should he have his 1st colonoscopy? (A) 30 years old (B) 35 years old (C) 40 years old (D) 45 years old (E) 50 years old

B. (The current recommendations are for patients to receive their first colonoscopy at age 50 years if there is no family history of colorectal cancer. If there is a family history of cancer in 1st-degree relative or multiple second-degree relatives, colonoscopy is recommended at 40 years of age, or 10 years before the age when the youngest first-degree relative was diagnosed with colon cancer (whichever is earlier). Repeat colonoscopy should be performed every 10 years. Patient nervousness should be dealt with by educational counseling by the doctor and does not change the recommended screening age.)

53-yo woman with history of polycystic kidney disease undergoes renal transplant from a cadaveric donor. She experiences several bouts of acute rejection that are controlled by appropriate increases in immunosuppression during 1st year posttransplant. During 4th posttransplant year her creatinine is rising gradually. Transcutaneous biopsy reveals architectural distortion and sclerosis of the renal tubules. What is the most likely outcome of the patient's condition? (A) Development of lymphoproliferative tumors requiring decreased immunosuppression (B) Gradual onset of renal failure and return to dialysis (C) Requirement of chronic antibiotic prophylaxis against urinary tract infection (D) Response to a short course of high-dose steroids (E) Response to increased chronic immunosuppression

B. (The patient is experiencing chronic rejection of her graft, a process of gradual decline in renal function. The underlying immune mechanism of chronic rejection is poorly understood but is assumed to be humoral in origin. Unfortunately, chronic rejection is unresponsive to steroids and other immunosuppressives, and it is the single most important cause of late graft failure. Factors implicated in chronic rejection are cadaveric source, past episodes of acute rejection, ischemic injury to the donor kidney, prolonged warm ischemic time prior to the transplant of the kidney, and poor compliance with drug therapy.)

66-yo postmenopausal white woman presents with a 1-month history of painless gross hematuria. She works as a hairdresser, is an avid coffee drinker, and has 50-pack-year smoking history. PE is normal. Which of the following would best confirm the diagnosis? (A) CT of the pelvis (B) Cystoscopy (C) Serum creatine kinase levels (D) Ultrasound (E) Urine culture

B. (The presence of otherwise unexplained hematuria in an individual over 40 denotes bladder cancer until proven otherwise. The patient also has numerous risk factors that raise suspicion for bladder cancer, such as her smoking history, possible exposure to dyes, and coffee drinking. Cystoscopy is the mainstay of diagnosis and staging of bladder cancer. A cystoscope is inserted into the bladder, and any tumor is then biopsied and resected)

10-yo girl is brought to pediatrician due to limp. She has been complaining of fatigue and pain in her right leg for months. Physical examination reveals multiple bruises on different sites of her body, enlarged anterior cervical and axillary lymph nodes, & hepatosplenomegaly. A blood smear is shown in the image. Which of the following is the best next step? (A) Call child protective services for suspected child abuse (B) Obtain a bone marrow biopsy (C) Obtain a liver biopsy (D) Perform a test for serum heterophile antibodies (E) Recommend a diet higher in vitamin C

B. (This child's symptoms of bone pain, easy bruising, and fatigue, as well as examination findings of hepatosplenomegaly and lymphadenopathy, suggest a malignancy, which is confirmed by the presence of blasts on the blood smear. The most common leukemia in children is ALL, and this diagnosis is supported by the lymphocytes on the smear. A bone marrow biopsy is recommended for all patients with ALL.)

33-yo man presents with complaints of *headaches and blurred vision* for past 3 months. He also notes *decreased libido*. Past medical history is significant for peptic ulcer disease, for which he takes omeprazole. Family history is significant for a mother with recurrent peptic ulcers and an uncle who died of a pancreatic tumor. PE is unremarkable. MRI of the brain reveals a mass in the pituitary. In addition to managing the pituitary mass, which of the following studies is most appropriate in this patient? (A) Calcitonin (B) Parathyroid hormone (C) Renal ultrasound (D) Thyroid ultrasound (E) Urinary metanephrines

B. (This patient has a pituitary mass causing headaches and blurred vision due to compression of the optic chiasm. It is most likely a prolactin-secreting adenoma because the patient is complaining of decreased libido, a common finding in men with hyperprolactinemia, along with possible impotence, infertility, gynecomastia, and galactorrhea. Given his history of peptic ulcer disease as well as a family history of peptic ulcer disease and pancreatic tumors, type I multiple endocrine neoplasia (MEN) should be considered in this patient since the autosomal dominant disorder consists of Pituitary tumors, Pancreatic tumors, and Primary hyperparathyroidism (the "3 P's"). Parathyroid hormone (PTH) as well as serum calcium and phosphate levels should be checked to evaluate for hyperparathyroidism and resultant hypercalcemia and hypophosphatemia)

23-yo African-American man presents to ER department with complaints of abdominal and bilateral lower extremity pain as well as chest pain that is worse on inspiration. He notes a frequent and long history of similar episodes throughout his life. T° is 101°F, RR is 25, and HR is 100/min. Lung examination reveals bronchial breath sounds and egophony at the left lung base. Oxygen saturation is 94% on 2-L nasal cannula. Lab tests show: WBC count: 12,000/mm³ Hemoglobin: 12 g/dL Hematocrit: 38% Platelet count: 200,000/mm³ Reticulocyte count: 2.1% What prophylactic treatment would have prevented this patient's acute chest pain? (A) Diphenyhydramine (B) Hydroxyurea (C) Pneumococcal vaccination (D) Prophylactic penicillin (E) Splenectomy

B. (This patient is suffering from acute chest syndrome as a result of sickle cell anemia. This is caused by sickling of his RBCs as a result of a substitution of valine for glutamine in the β-globin chain of hemoglobin. Sickling is prompted by hypoxia, acidosis, dehydration, or variations in body temperature. Acute chest syndrome is a vasoocclusive crisis within the lungs associated with infection of pulmonary infarction. Affected patients present with hypoxia, fever, dyspnea, chest pain, and progressive pulmonary infiltrates seen on x-ray studies. Such episodes can be prevented by hydroxyurea, which decreases the incidence of sickle cell crises. Hydroxyurea acts as an inhibitor of deoxynucleotide synthesis, which reduces the production of RBCs containing high level of sickle hemoglobin and favors the production of RBCs containing a high fetal hemoglobin level)

56-year-old man presents to his physician complaining of severe fatigue. He began to feel increasingly tired about 6 months ago, but believes that his fatigue has been worsening over the past 3 weeks. He also notes he has had a nonproductive cough for about 2 weeks and has experienced several episodes of drenching night sweats. On examination he has several large bruises on his extremities but recalls no injuries. Abdominal examination reveals massive enlargement of both the liver and the spleen, without any lymphadenopathy. Laboratory studies show: WBC count: 1200/mm3; Neutrophils: 58%; Eosinophils: 7% Lymphocytes: 30%; Monocytes: 0%; Basophils: 5% RBC count: 3.0/mm; Hemoglobin: 7.5 mg/dL Platelet count: 18,000/mm3 Peripheral blood smear reveals irregular nuclei and cell membranes, as well as cytoplasmic projections. Which of the following is the most likely diagnosis? (A) Acute lymphocytic leukemia (B) Hairy cell leukemia (C) Idiopathic thrombocytopenic purpura (D) Infectious mononucleosis (E) Nodular sclerosing Hodgkin's lymphoma

B.(Hairy cell leukemia is a rare malignancy of B lymphocytes. It is most common in patients 50-60 years old, and shows a 5:1 male predominance. Patients generally present with the symptoms of pancytopenia, namely fatigue, easy bruising, and recurrent infection, as well as splenomegaly (and hepatomegaly in 50% of patients). Mycobacterial infections are common due to a striking monocytopenia that is nearly universal in these patients. Lymphadenopathy is rare. Examination of the spleen shows infiltration of the red pulp by these cells, which demonstrate the characteristic "hairy cell" cytoplasmic projections and are typically tartrate-resistant acid phosphatase positive. Although patients may demonstrate markedly abnormal blood cell counts at presentation, >90% of patients will live >10 years with treatment)

Which of the laboratory findings in the table below are characteristic of hemophilia B? PT refers to prothrombin time and aPTT refers to activated partial thromboplastin time. (A) A (B) B (C) C (D) D (E) E

B.(This profile is consistent with hemophilia B. Hemophilia B (Christmas disease) is an X-linked recessive coagulopathy involving a deficiency of coagulation factor IX that may lead to mild to severe bleeding, depending on the factor activity. A prolonged activated partial thromboplastin time is seen with hemophilia. Prothrombin time, bleeding time, and platelet count are expected to be normal in a patient with hemophilia

Which of the laboratory findings in the table below are characteristic of hemophilia A? PT refers to prothrombin time and aPTT refers to activated partial thromboplastin time. (A) A (B) B (C) C (D) D (E) E

C (This profile is consistent with hemophilia A. Hemophilia A is an X-linked recessive coagulopathy involving a deficiency in coagulation factor VIII that may lead to mild to severe bleeding, depending on the factor activity. A prolonged activated partial thromboplastin time is seen with hemophilia. Prothrombin time, bleeding time, and platelet count are expected to be normal in a patient with hemophilia)

42-year-old woman who smokes tobacco is found to have acute-onset respiratory distress and tachycardia 4 hours after a non-emergent cholecystectomy. She is subsequently treated for a symptomatic pulmonary embolism. She is begun on low-molecular-weight heparin and warfarin while in the hospital, and is supplied with subcutaneous doses of low-molecularweight heparin to take at home for a total course of 5 days, in addition to the warfarin that she will take for at least 6 months. The initial 5 days of overlap of both heparin and warfarin is necessary because at the beginning of treatment, warfarin actually leads to hypercoagulability. What is the underlying reason for this? (A) There is an initial increase in vitamin K-dependent coagulation factors (B) Venous valvular insufficiency is exacerbated during the first 3 days of warfarin therapy (C) Warfarin causes a more rapid drop in the levels of proteins C and S than factors II, VII, IX, and X (D) Warfarin induces resistance of factor V to degradation by activated protein C (E) Warfarin leads to an initial increase in platelet aggregation

C. (Along with the coagulation factors II, VII, IX, and X, the anticoagulant proteins C and S are also vitamin K dependent, and therefore affected by warfarin, which is a vitamin K analog. Proteins C and S have shorter half-lives than the coagulation factors, and therefore a faster turnover in the body. Thus, for a brief window of approximately 3 days after the initiation of warfarin therapy, the body has normal function of its coagulation factors, but loss of anticoagulant proteins C and S, rendering the patient hypercoagulable. Negative consequences of this are avoided by administering heparin with warfarin for the first 5 days)

69-yo woman has been in ICU for 7 days following complicated hip replacement surgery. She is currently receiving heparin subQ and wears intermittent pneumatic compression devices on her lower extremities bilaterally. She has developed new-onset right calf pain, edema, tenderness, and a positive Homans' sign. A Doppler ultrasound revealed a deep vein thrombosis. Her platelet count is 78,000/mm³, and there has been no evidence of spontaneous bleeding. Which of the following will best help prevent future complications? (A) Beginning warfarin therapy (B) Discontinuation of bilateral pneumatic compression devices (C) Discontinuation of heparin (D) Performing venography (E) Placing an inferior vena cava filter (F) Transfusing platelets

C. (The patient in this clinical scenario most likely has heparin-induced thrombocytopenia (HIT), which commonly occurs within 4-10 days after the initiation of unfractionated heparin treatment. Unlike other drug-induced thrombocytopenia, HIT is associated with thrombosis due to platelet activation rather than bleeding. Because platelet counts typically remain >20,000/mm³, spontaneous bleeding is rarely seen. Pathophysiology of this disorder includes the presence of platelet antibodies that cause limb- and life-threatening thrombosis, often at unusual sites including arteries. The first intervention in cases of HIT is immediate cessation of any heparin and immediate initiation of a direct thrombin inhibitor such as argatroban or lepirudin)

2-yo girl presents to the pediatrician because her mother noticed a mass while dressing her. Aside from the mass, the child's mother has no concerns. She reports that the child is otherwise well and denies any fever, weight loss, or fatigue. The child's temperature is 37°C (98.6°F), blood pressure is 100/60 mm Hg, heart rate is 80/min, and respiratory rate is 25/min. A smooth, firm, left-sided nontender flank mass is palpated. There is no hepatomegaly, no splenomegaly, and no lymphadenopathy. A CT scan reveals only an encapsulated left-sided mass and no other abnormalities. Laboratory studies show: BUN: 8 mg/dL; Cr: 0.4 mg/dL Alanine aminotransferase: 20 U/L; Aspartate aminotransferase: 20 U/L; Total bilirubin: 0.4 mg/dL Alkaline phosphatase: 70 U/L; Urine vanillymandelic acid: Negative; WBC count: 6200; Hgb: 14; Platelet count: 190,000/mm3; Urine RBCs: 1+; Urine protein: 0 Urine WBC esterase: Negative Which of the following is most appropriate therapy? (A) Chemotherapy only (B) Local excision of tumor without nephrectomy (C) Nephrectomy and chemotherapy (D) Nephrectomy only (E) Radiation

C. (This child has Wilms' tumor. Wilms' tumor usually presents between 2 and 4 years of age with a painless flank mass that does not cross the midline. Some children, such as this one, also present with hypertension and microscopic hematuria. Others can present with fever, weight loss, polyuria, dysuria, bone pain, nausea, and vomiting. The diagnosis is made by abdominal CT. Stage 1 is treated with nephrectomy and chemotherapy. If metastasis is present, radiation therapy is added. This child has no evidence of metastasis.)

55-yo woman presents with complaints of pain in her hands in the cold. On further questioning she says she notices that her hands have a bluish hue when she is outside, and that they become pale and then quite red when warm. Her physician, concerned that Raynaud's phenomenon may indicate the presence of an autoimmune illness, initiates an autoimmune work-up that is entirely negative. 3 weeks later she returns to report of visual "floaters." She states she feels fine otherwise. Her PE is negative aside from several large bruises on her legs. Which of the following is the most appropriate management? (A) Begin a course of prednisone to alleviate the patient's autoimmune symptoms in the absence of concrete diagnosis (B) Discuss the possibility of domestic violence with the patient (C) Order plasma electrophoresis (D) Reassure the patient and reevaluate in 3 months (E) Resend the patient's serum for antinuclear antibody tests

C. (This patient has Waldenström's macroglobulinemia, a lowgrade malignant lymphoma of plasmacytoid lymphocytes that secrete excessive amounts of IgM. Most patients with this disease present with symptoms of hyperviscosity (Raynaud's phenomenon and visual disturbances), and protein-protein interaction (platelet dysfunction). Diagnosis requires the demonstration of an IgM spike on plasma electrophoresis, as well as infiltration of bone marrow with plasmacytoid lymphocytes. Plasmapheresis is used to control hyperviscosity initially because response to chemotherapy can take several weeks. Treatment regimens involve the same chemotherapeutics as those used for multiple myeloma, including alkylating agents and prednisone. Overall mean survival is 5 years)

56-yow with diabetes and proteinuria complaining of right groin pain and decreased urine output for the past day. She denies trauma, fever, or history of kidney stones or UTIs. Her T° is 99.0°F, BP is 146/90; pulse is 104, RR is 16. Heart and lung examinations are normal; she has no visible rashes and no costovertebral angle tenderness. There is trace ankle edema bilaterally. Lab tests show: Na: 134; K: 4.9; Cl: 100; HCO3: 24; BUN: 38 mg/dL; Cr: 1.6 mg/dL; Albumin: 2.2 g/dL; Urinalysis: 3+ blood, 4+ protein, no WBCs, no stones. What is the most appropriate next step in management of this patient's condition? (A) Analgesics and angiotensin-converting enzyme inhibitors (B) Kidney biopsy and high-dose corticosteroids (C) Renal vein ultrasound and heparin (D) Thiazides and spiral CT of the pelvis (E) Urine culture and oral quinolones

C. (This patient has diabetes with proteinuria and likely has nephrotic syndrome. Among the many sequelae of nephrotic syndrome is hypercoagulability secondary to the loss of antithrombotic proteins in the urine: proteins C and S and antithrombin. These patients are at increased risk for arterial and venous thrombotic complications, including myocardial infarction, pulmonary embolism, deep vein thrombosis, and, as is the case with this patient, unilateral renal vein thrombosis. Classically, patients with renal vein thrombosis present with flank pain (secondary to distention of the renal capsule), hematuria, and evidence of renal impairment. Timely diagnosis via renal ultrasound, CT, or direct venography as well as treatment with heparin are crucial, as the disruption of renal blood flow can lead to permanent damage)

68-yo woman presents for a routine visit. She is generally in good health, her only medications are hydrochlorothiazide and metoprolol for high BP. She has had no recent changes in her health, and review of systems is negative. Examination shows a few enlarged cervical and inguinal lymph nodes. The liver and spleen are not enlarged. Electrolyte panel is within normal limits, a peripheral smear shows smudge cells, and CBC shows a WBC count of 47,000/mm³ (with 89% lymphocytes), hemoglobin level of 12.9 g/dL, and platelet count of 213,000/mm³. Which of the following is the most appropriate management? (A) Chemotherapy (B) Imatinib therapy (C) No treatment is indicated at this time (D) Radiation therapy (E) Splenectomy

C. (This patient most likely has chronic lymphocytic leukemia(CLL), which mainly affects individuals >60 years old and accounts for 30% of all leukemias in USA. The patients are often asymptomatic at presentation, and the disease is only detected on laboratory tests. In this case, the patient has an increased WBC count consisting predominantly of lymphocytes. This patient has a predicted median life expectancy of 7 years. Early treatment does not improve the outcome, and management is palliative rather than aimed at a cure. Because this patient is asymptomatic, no treatment is indicated at this time)

97-year-old man was admitted to the hospital because of weakness and rectal bleeding for the previous 24 hours. Questioning reveals a decrease in stool caliber over the past several months. Examination reveals a nontender mass in LLQ. Stool heme is positive. Serum hemoglobin is 8.8 g/dL. Further work-up reveals colon cancer, Duke stage D. The patient's spouse and only child are no longer living, but the patient's 50-year-old granddaughter pleads with the physician not to inform her grandfather of the diagnosis, stating that he is an anxious man and that she wants him to enjoy his remaining time. Which of the following is the most appropriate next step? (A) Agree not to reveal the diagnosis to the patient (B) Ask the granddaughter to obtain other family members' opinions and try to reach consensus (C) Ask the patient how much information he wants to know (D) Request a consult from the hospital's ethics team (E) Request psychiatric evaluation of the patient

C. (With rare exceptions, patients are entitled to know their diagnosis and prognosis, even if they are old, their condition is probably terminal, and they have anxious personalities and/or psychiatric diagnoses. Exceptions to the physician's obligation to discuss the diagnosis with the patient include the previous request of the patient to not be informed or the belief of the physician that disclosure would severely harm the patient)

Typically affects patients older than 50 years. It is a myeloproliferative disorder that is often discovered on routine blood tests where leukocytosis and thrombocytosis are discovered. When symptomatic, fatigue, weight loss, malaise, abdominal discomfort, and bleeding due to platelet dysfunction are common. The t(9;22) translocation creates the Philadelphia chromosome, which is often seen.

CML

This sign refers to ecchymosis in the periumbilical region suggestive of retroperitoneal hemorrhage in severe acute pancreatitis.

Cullen's

9-yo African-American boy is brought to the clinic by his mother after she noticed a droop on the left side of his face and difficulty moving his left arm. There was no preceding head trauma or aura, and he was conscious throughout. The deficit lasted for about 3 hours and he returned to baseline activity except for some residual weakness of the left hand. He has a maternal grandfather who had a heart attack at age 63 years, and the child's father has hypertension. Review of systems is significant for easy fatiguing with exercise, but negative for fevers, loss of appetite, weight loss, vomiting, or diarrhea. Significant findings on examination include mild pallor and modestly decreased left hand strength. Lab studies show his total cholesterol level is 140 mg/ dL. Which of the following diagnoses could unify these findings? (A) Familial hypercholesterolemia (B) Hypertension (C) Multiple myeloma (D) Sickle cell disease (E) Simple partial seizure

D. (A stroke or transient ischemic attack in a child should prompt a search for causes other than those commonly seen in adults. Sickle cell disease is often thought of in African-Americans, but should also be considered in individuals from Central or South America. Approximately 11% of children with sickle cell disease have a stroke before the age of 20 years, and transient ischemic attacks can often be the first sign of an impending stroke. The pallor and fatiguing can also be clues pointing toward anemia. Chronic red blood cell transfusion in sickle cell patients has been shown to reduce the risk of cerebral thrombotic events as well as pain crises and acute chest syndrome)

67-yo woman is seen for *cough productive of bloody sputum and 25-lb unintentional weight loss, both occurring within the past 6 months*. In addition, the patient notes that over past 3 months she has become increasingly *lethargic and experienced bouts of nausea*. She has *smoked two packs per day for past 50 years*.She denies a history of heart failure or liver cirrhosis. She currently takes no medications. Her temperature is 36.7°C, BP is 125/85 mm Hg, pulse is 78/min and regular, RR 15/min, and oxygen saturation is 99% on room air. Examination reveals *crackles at the left lower lung* field; no lower extremity edema is present. Laboratory test show: WBC count: 6000/mm3; Hgb: 14.7 g/dL; Platelet count: 210,000/mm3; Na+: 125 mEq/L; K+: 4 mEq/L; Cl−: 102 mEq/L CO2: 24 mmol/L; BUN: 8 mg/dL; Cr: 1 mg/dL; Glucose: 120 mg/dL; Urine osmolality: 125 mOsm/kg; Urinary Na+: 35 mEq/L; X-ray of the chest shows a focal 5*-cm mass lesion* in the right lower lung that is corroborated by CT. Which of the following is the most likely histologic type of lung cancer present in this patient? (A) Adenocarcinoma (B) Bronchoalveolar cell carcinoma (C) Large cell carcinoma (D) Small cell carcinoma (E) Squamous cell carcinoma

D. (An 11.3-kg (25-lb) unintentional weight loss, hemoptysis, lesion visible on CT scan, and a significant smoking history should raise concern for lung cancer. The patient is suffering from euvolemic hyponatremia due to syndrome of inappropriate ADH secretion (SIADH), a paraneoplastic syndrome seen in small cell carcinoma of the lung. Small cell carcinoma, along with sarcoidosis, pneumonia, head injury, and antipsychotics or antidepressants can cause nonosmotically stimulated release of ADH, resulting in euvolemic hyponatremia. Diagnosis is based on urine osmolality >50-100 mOsm/kg with concurrent serum hyposmolality in the absence of a physiologic reason for increased ADH (chronic heart failure or cirrhosis). Urine sodium >20 mEq/L is used to show that the patient is not hypovolemic. Of the bronchogenic carcinomas of the lung, small cell carcinoma carries the worst prognosis and is assumed to be metastatic at time of diagnosis)

62-yo woman has an abnormal Pap smear finding of low-grade squamous intraepithelial lesion (LSIL) on routine screening. She does not have a significant past medical history, and she is immunocompetent. Her last Pap smear 3 years ago was normal. She has never had an abnormal Pap smear and has always complied with obtaining routine screening tests. She is entirely asymptomatic and postmenopausal. She is referred for colposcopy, and biopsy of a satisfactory specimen confirms LSIL. What is the most appropriate next step in the management? (A) Endocervical curettage (B) Loop electrosurgical excision procedure (C) Observation consisting of a repeat Pap smear in 1 year (D) Observation consisting of colposcopy and repeat Pap smears every 4 months for up to a year (E) Repeat colposcopy in 1 year, even if repeat Pap smear is normal

D. (Recent evidence suggests that most low-grade squamous intraepithelial lesions will regress spontaneously. Current guidelines indicate observation of a biopsy-confirmed LSIL is appropriate management for a reliable patient that is also immunocompetent)

6-yo African-American girl presents with intense pain in both feet after jumping in cold swimming pool. There was no trauma reported, pain seems to decrease with warming of feet. PE show scleral icterus and splenomegaly. There is no point tenderness of the ankles or feet, but the toes are cool and appear dusky. CBC shows Hbg 10.2 g/dL, and a liver panel shows increased indirect bilirubin. A peripheral smear is shown in the image. Which of the following long-term treatments is needed to minimize the recurrence of her condition? (A) Avoidance of sulfa drugs and fava beans (B) Corticosteroids (C) Hematopoietic stem cell transplant (D) Hydroxyurea (E) Serial transfusions

D. (Sickle cell anemia is a recessive genetic disease that often presents with painful microocclusive crises due to sickling of RBCs in capillaries. Chronic hemolysis is evident with jaundice and elevated unconjugated bilirubin. Blood smear shows sickle shaped RBCs and nuclear fragments called Howell-Jolly bodies. Acute treatment is symptomatic pain control, increasing oxygen tension to reduce future sickling (oxygen), and reducing the occlusive crisis through hydration. Long-term treatment includes hydroxyurea. Hydroxyurea stimulates the production of fetal hemoglobin, which will not sickle with low oxygen tension)

A 58-yo man with a history of psoriasis presents for his annual examination. He was first diagnosed with psoriasis 3 years ago and has shown little improvement despite numerous therapeutic regimens. His plaques are primarily distributed around his waist and buttocks and have recently begun to itch. Skin biopsy reveals atypical lymphocytes aligned along basal layer and epidermal microabscesses (see image). Which of the following is the most likely diagnosis? (A) Contact dermatitis (B) Eczema (C) Inverse psoriasis (D) Mycosis fungoides (E) Pityriasis rosea

D. (The arrow in the image indicates an epidermal cluster of atypical lymphocytes (Pautrier's abscess). This is pathognomonic for mycosis fungoides, a subtype of cutaneous T-lymphocyte lymphoma (CTCL) that is unrelated to fungal infection in spite of the name. This disease is most frequently found in men in their mid-50s and is often misdiagnosed for years. Skin lesions may progress from patch phase to plaque phase to cutaneous tumor, and subcutaneous deposits in the face may lead to the classic (though rarely seen) "leonine facies." Sézary syndrome is mycosis fungoides with erythroderma, lymphadenopathy, and the presence of atypical T lymphocytes in the blood)

23-year-old woman is diagnosed with stage IIIA Hodgkin's lymphoma after an extensive work-up. As her physician is explaining the recommended treatment regimen for her disease, the patient confesses she has been hoping to become pregnant in the next few years. However, she states she is willing to wait until she has completed her treatment. She wants to know the consequences of both her primary disease of Hodgkin's lymphoma and her treatment regimen, as well as what effects they may have on a potential pregnancy. Which of the following complications is the LEAST likely? (A) An increased risk of second malignancy (B) Ovarian failure that will preclude pregnancy (C) Pericarditis that may cause congestive heart failure during the pregnancy (D) Recurrence of the patient's disease due to increased estrogen (E) Restrictive lung disease that may be exacerbated by pregnancy

D. (The patient is presenting with stage IIIA disease, which implies that her disease has spread to nodal sites on both sides of her diaphragm. This disease stage will require therapy with both systemic chemotherapy and radiation to the mediastinum, and potentially the abdomen and pelvis. This patient should be concerned about many effects of her disease and its treatment that may prevent or complicate her pregnancy; however, there is no evidence that the hormonal milieu of pregnancy promotes recurrence of Hodgkin's disease. Importantly, this type of evidence does exist in breast cancer, where increased estrogen may promote the growth of tumor cells)

14-month- boy with no known medical history presents because of a black eye. He has had the injury for at least a week, but his mother is uncertain as to when or how it happened, although she does report that he has had a low-grade fever and has been sleeping "all the time" for the past 6 days. On examination he is pale and ill appearing with a temperature of 38°C (100.4°F), BP of 120/72, HR 86/min, RR of 22/min. He is in 10th percentile for weight, the 10th percentile for height, and 50th percentile for head circumference. There is periorbital bruising and proptosis of the right eye, petechiae on his legs, and cervical lymphadenopathy. No hepatomegaly, splenomegaly, or palpable masses. Lab tests show: Blood urea nitrogen: 7 mg/dL Creatinine: 0.4 mg/dL WBC count: 1200/mm3 Hemoglobin: 8 g/dL Platelet count: 19,000/mm3 Activated partial thromboplastin time: 25 sec Prothrombin time: 23 sec Alanine aminotransferase: 16 U/L Aspartate aminotransferase: 14 U/L Urine vanillylmandelic acid: Elevated D-dimer: Normal Which of the following is the most likely diagnosis? (A) Acute lymphocytic leukemia (B) Aplastic anemia (C) Child abuse (D) Neuroblastoma (E) Pheochromocytoma

D. (This child has metastatic neuroblastoma, which is common in children <2 years old. Primary neuroblastoma most commonly presents as a painless abdominal mass, hypertension, respiratory distress, Horner's syndrome, or cord compression. Often, however, neuroblastoma is not diagnosed until after it has metastasized. Tumor infiltration of the bone marrow causes pancytopenia. Tumor infiltration of the periorbital bones causes periorbital ecchymoses and proptosis, an appearance often referred to as "raccoon eyes." Low-grade fever, fatigue, and failure to thrive (which is evidenced by his small size) occur with metastatic disease. Urinary catecholamines are elevated in neuroblastoma, but definitive diagnosis requires biopsy. Treatment involves chemotherapy and surgical excision of local disease. Prognosis varies with age at presentation: infants with metastatic disease respond favorably to treatment, while most children >1 year with advanced neuroblastoma will die from progressive disease despite intensive multimodality therapy)

32-yow develops progressive idiopathic cardiomyopathy and eventually undergoes a cardiac transplant. She is placed on rigorous protocol for immunosuppression. 2 years later she develops right-sided sinus fullness, and imaging reveals a mass filling the right paranasal sinus. She is treated with a reduction in immunosuppression and acyclovir. She achieves a full recovery. Why is acyclovir part of her therapeutic regimen? (A) Acyclovir has also been shown to have antineoplastic properties (B) Acyclovir is necessary for prophylaxis against cytomegalovirus in any immunosuppressed patient (C) Acyclovir will reduce the risk of infection due to radiation of the mucous membranes and subsequent breakdown (D) Eradication of Epstein-Barr virus may contribute to resolution of the paranasal sinus mass (E) Since the patient's immunosuppressive regimen must be decreased, protection against viruses that might infect the patient's new heart is particularly important

D. (This patient has developed a posttransplant lymphoproliferative disorder (PTLD). Any patient who is chronically immunosuppressed is at risk for development of lymphoproliferative malignancy, and a cardiac transplant patient is at intermediate risk (4.6% incidence) compared to kidney (1.0%) or liver (3.0%) transplant patients. PTLDs generally occur in extranodal locations, and may respond completely to reduction in immunosuppression; however, since immunosuppression is necessary for protection of grafted organs, radiotherapy is also often used. In addition, most PTLDs are related to primary or reactivation infection with Epstein-Barr virus. Some evidence demonstrates that the use of acyclovir during treatment for PTLD contributes to eradication of the virus and resolution of the malignancy caused by it. The majority of centers thus employ acyclovir as an adjuvant therapy to radiotherapy/surgery coupled with a reduction in immunosuppression.)

Which of the laboratory findings in the table below are characteristic of thrombocytopenia? PT refers to prothrombin time and aPTT refers to activated partial thromboplastin time. (A) A (B) B (C) C (D) D (E) E

D. (This profile is consistent with thrombocytopenia, as seen with disorders such as idiopathic thrombocytopenic purpura (ITP) or HIT)

6-yo boy presents with 5 days of nosebleeds and a rash. The nosebleeds have increased in frequency to three times a day. The mother reports no fevers at home, though he did have 4-5 days of cough and runny nose 2 weeks ago. His past medical history is unremarkable and he takes no medications. On PE he is afebrile with petechial rash and some crusted blood on the nares. No splenomegaly. CBC shows a platelet count of 127,000/mm³ and normal Hgb. A peripheral blood smear shows megathrombocytes. Initial treatment should consist of which of the following? (A) Corticosteroids (B) Dialysis (C) Intravenous gamma-globulin (D) Observation (E) Platelet transfusion

D. (idiopathic thrombocytopenic purpura (ITP) is a disorder of primary immune platelet destruction. The clinical presentation is of insidious onset of mucocutaneous bleeding. The diagnosis is one of exclusion; CBC usually shows isolated thrombocytopenia, and large platelets may be apparent on peripheral smear. Other tests may include a bone marrow biopsy to exclude aplastic anemia or drug-induced suppression of megakaryocytes. In children, this disease is usually self-limiting and requires no treatment, but adults generally require immunosuppression or dialysis or surgical (through splenectomy) management. Children with chronic ITP have platelet counts from 20,000-75,000/mm³ and typically do not require treatment. Pulse or short-course corticosteroids may be used in refractory cases, but long-term daily steroid use should be avoided)

37-yo woman gives birth to 3rd healthy child. He is discharged from hospital with her at 2 days of life. For the first 3 months of his life, however, his parents note poor weight gain, chronic diarrhea, and persistent diaper rash. CBC reveals paucity of lymphocytes, and antibody staining demonstrates deficiency of X-linked interleukin-2 receptor chain. Baby is diagnosed with X-linked severe combined immunodeficiency (SCID). His parents are told that the standard of care for him is a bone marrow transplant from a major histocompatibility complex-matched related donor. His two sisters are tested and are not matches. His parents are interested in exploring the option of gene therapy. Which of following concerns is unique to the use of gene therapy for tx of SCID? (A) Graft-versus-host disease (B) Increased risk of failure (C) Increased susceptibility to viral illness in the future (D) Inefficient response to vaccines in the future (E) Leukemia

E. (Patients with X-linked SCID and no matched related donor are very good candidates for stem cell gene therapy. The defect in the X-linked interleukin-2 receptor chain gene is known, making this disease a prototype for gene treatment. The first gene therapy in patients with SCID was performed in 1991, and involved transduction with a retroviral vector with clinical improvements documented in all patients. Trials have continued and several patients have been successfully treated in this fashion. A significant number, however, have developed a rapidly fatal leukemia, a transformation thought to result from the gene insertion into patient cells with oncogenic action)

28-yo man presents to clinic complaining of heaviness in his testicle for 2 weeks. He says he feels as though his testicle is enlarged. The man has a temperature of 37.2°C, HR of 60/min, and BP of 115/70 mm Hg. He has normal abdominal exam with no palpable masses. The right testicle is noticeably larger than the left testicle. There are no discrete nodules. Testicular ultrasound is performed, followed by an orchiectomy. He is found to have a seminoma and a retroperitoneal lymph node that is enlarged at 1.8 cm. He is given a diagnosis of *stage IIA testicular seminoma* (T2N1M0). What additional tx needed? (A) Contralateral orchiectomy (B) Platinum-based chemotherapy and bilateral orchiectomy (C) Prophylactic mediastinal radiation (D) Retroperitoneal lymph node dissection (E) Retroperitoneal radiation

E. (Seminoma is a tumor type that is extremely sensitive to radiation. Stages I or IIA disease can be successfully treated with orchiectomy and irradiation of the retroperitoneum. The low dose of radiation that is required is usually well-tolerated with minimal gastrointestinal side effects. Chemotherapy can be used as salvage therapy for patients who relapse following irradiation. The staging of seminomas is as follows: stage I: disease confined to the testis; stage II: retroperitoneal nodal involvement (IIA if <2 cm and IIB if >2 cm); stage III: supradiaphragmatic nodal involvement or involvement of the viscera)

69-yo man with CHF and chronic obstructive pulmonary disease has palpable adenopathy in the neck and inguinal region. He feels perfectly well and has normal blood counts. Lymph node biopsy shows follicular small cleaved lymphocytic lymphoma. Bone marrow biopsy shows peritrabecular infiltrates of small cleaved lymphocytes. Which of the following is the best management? (A) Arrange for bone marrow transplant (B) Arrange for immediate hospice care (C) Begin palliative radiation therapy to involved lymph node beds (D) Initiate an aggressive multiagent chemotherapy regimen (E) Manage symptoms conservatively with close follow-up

E. (The patient has been diagnosed with follicular lymphoma, a low-grade disease of B-lymphocyte origin that is a result of a t(14;18) translocation. Such disease is almost always stage III (involved nodes above and below the diaphragm) or stage IV(disseminated disease) at diagnosis, as was the case for this patient. Rarely, low-grade lymphomas are curable with intensive chemotherapy; however, they are generally indolent, with most patients surviving 7 to 10 years with no therapy. The "watch and wait" approach has become the standard of care for patients with low-grade lymphomas, with palliative chemotherapy started only on progression of disease. Histologic transformation may occur in 30%-50% of follicular lymphomas to a large B-lymphocyte lymphoma. Median survival is <1 year after transformation occurs)

45-yo man presents to his primary care with complaint of patchy dry areas on his skin. After several trials of skin emollients and corticosteroid without improvement, the lesions are biopsied by a dermatologist. Pathologic examination reveals a population of *atypical mononuclear cells infiltrating the dermis and epidermis*. CBC is normal, and no atypical cells are observed on peripheral blood smear. The patient states that he feels quite well. Which of the following is most appropriate as recommended by the patient's dermatologist? (A) Anticipation of bone marrow transplant (B) Observation alone (C) Systemic chemotherapy (D) Systemic corticosteroids (E) Treatment of the affected areas of skin with topical nitrogen mustard (F) Treatment of the entire skin surface via total skin electron beam therapy

E. (The patient is presenting with cutaneous T-cell lymphoma (CTCL), or mycosis fungoides. This disease is a T-lymphocyte lymphoma that is generally indolent. CTCL may affect patients in several different stages, although one stage does not necessarily lead to another. Patients may live for 20+ years without progression of disease. The phases of disease are as follows: The phase of least severity is a patch stage, involving only the epidermis in discrete areas of the skin. This phase may or may not progress to a plaque stage, affecting discrete skin areas but invading the dermis as well as the epidermis.)

65-yo previously healthy Ashkenazi Jewish woman presents with complaints of *tenderness throughout her ribs and spine*. She has noted *increasing fatigue and weight loss* over past 6 months, and has missed several days of work due to minor infections. PE is within normal limits aside from point tenderness near her posterior ribs and thoracic spine. Laboratory studies reveal a WBC count of 8400/mm³, hemoglobin level of 7.9 g/dL, platelet count of 200,000/mm³, and 3+ protein in the urine. X-ray of the chest reveals lytic lesions in several areas of the ribs and spine. Bone scan is within normal limits. What is the most likely pathology behind her fractures? (A) Anorexia nervosa (B) Bony infarcts resulting from Gaucher's disease (C) Congenital osteoporosis (D) Domestic abuse (E) Increased osteoclastic activity secondary to multiple myeloma (F) Metastatic disease to bone from an unidentified breast cancer

E. (The patient's most likely diagnosis is multiple myeloma. This malignancy of plasma cells leads to proliferation of a single plasma cell clone. This plasmacytosis leads to secretion of osteoclast-activating factor by myeloma cells, increasing osteoclastic activity and causing lytic lesions. The plasmacytosis also contributes to immunosuppression (because one antibody clone dominates rather than the hundreds of thousands that are found in a normal patient) and anemia. The proliferation of plasma cells also causes a proliferation of antibody clones that spill into the urine, known as Bence Jones proteins, and may eventually cause renal failure.)

53-yow presents with diffuse back and rib pain that is worsened with movement. Her periods have recently stopped, but denies experiencing hot flashes or other menopausal symptoms. She also notes 15-lb weight gain over the past 3-4 months. On further questioning she states she has noted tingling in her fingers and toes for the past 7 months, and has recently begun tripping frequently and having trouble opening doors and buttoning her shirts. PE reveals diffuse lymphadenopathy, abd exam reveals hepatomegaly. Her skin is warm and dry;hyperpigmentation is noted in regions of neck and shoulders, and her digits are noted to be clubbed. Laboratory testing and peripheral blood smear results reveal large numbers of plasma cells; additional laboratory findings are: WBC count: 13,000; Hgb: 9.8; Platelet count: 340,000/mm3 Erythrocyte sedimentation rate: 64 mm/hr; BUN: 35;Cr: 2.1; Thyroid-stimulating hormone: 25 U/mL; Blood glucose level: 384 mg/dL; Ca2+: 15.4 mg/dL; Which of the following is most likely to confirm the patient's underlying diagnosis? (A) Complete thyroid evaluation (B) Dexamethasone suppression test (C) Hemoglobin A1c (D) Pulse oximetry (E) Urine and serum protein electrophoresis (F) WBC differential

E. (This patient is presenting with POEMS syndrome, a constellation of symptoms associated with osteosclerotic multiple myeloma. Symptoms include Polyneuropathy, Organomegaly, Endocrinopathy, Multiple myeloma, and Skin changes. Polyneuropathies are generally progressive sensorimotor deficits. Hepatosplenomegaly is generally seen, in contrast to other myeloma patients. Endocrine manifestations include amenorrhea, diabetes mellitus, hypothyroidism, and adrenal insufficiency. Skin changes are diverse, but may include hyperpigmentation, hypertrichosis, thickening, and digital clubbing. Although this patient's symptoms seem unrelated, her peripheral blood smear demonstrates a large number of plasma cells, as are seen in multiple myeloma. In addition, her laboratory work reveals anemia, hyperuricemia, and hypercalcemia, all consistent with a diagnosis of multiple myeloma. This diagnosis can be confirmed by the presence of an M spike on protein electrophoresis of urine and serum.)

18-yo college freshman presents to ER department after suffering a seizure at a party. She is accompanied by her friend, who states that the patient had been consuming alcohol and had complained of severe abdominal pain and vomited several times before she began convulsing. The patient is admitted for observation and work-up of her symptoms. She has no further seizures and appears to be recovering well. The next morning an observant medical student notes that the urine collecting in her Foley catheter bag is reddish-brown. The student double-checks the patient's laboratory results and finds that she has a positive urine toxicology screen that could explain her symptoms. Which of the following drugs most likely contributed to patient's symptoms? (A) Cocaine (B) Heroin (C) Marijuana (D) Methamphetamine (E) Phenobarbital

E. (This patient is presenting with classic symptoms of acute intermittent porphyria (AIP): gastrointestinal distress and neurological symptoms following ingestion of alcohol and a barbiturate, followed by production of brown or reddish urine. AIP is a severe autosomal dominant form of porphyria, which results from a partial deficiency of porphobilinogen deaminase activity, which is the third enzyme in the pathway of heme synthesis. Attacks occur when intermediates in the heme synthetic pathway build up, and result in a variety of symptoms including GI distress, tachycardia, hypertension, peripheral neuropathy, seizures, and psychiatric symptoms. Attacks can be triggered by stress, alcohol consumption, the premenstrual period, and consumption of drugs that are cytochrome 450 (CYP450) inducers, such as barbiturates, because they increase the demand for heme synthesis. After acute attacks urine can appear to be brown or reddish. Many patients with AIP have a family history of the disorder)

55-yow who was successfully treated with chemotherapy for breast cancer several years ago presents complaining of 2 months of easy bruising, bleeding gums, fatigue, and shortness of breath. On PE she has a low-grade fever and several bruises in different locations on her body. She does not have JVD. No masses are palpable on breast exam. Heart and lung examination are within normal limits. Abdominal examination is significant for hepatosplenomegaly. Her WBC count is 35,000/mm³. Her WBCs are myeloperoxidase positive. Which of the following statements regarding patients carrying this diagnosis is true? (A) Incidence decreases with increasing age (B) Previous chemotherapy does not affect risk for developing the disease (C) Prognosis is not affected by the cytogenetics of the disease (D) Retinoic acid was once used to treat this disease but is no longer part of the current treatment (E) They are at increased risk for stroke

E. (This patient presents with a thrombocytopenic picture and with leukocytosis. Her positive myeloperoxidase test is diagnostic for AML. Complications of this disease include leukostasis leading to hyperviscosity retinopathy, transient ischemic attacks/cerebrovascular accidents, tumor lysis syndrome,while undergoing treatment, gingival hyperplasia, central nervous system involvement, recurrent infection, and disseminated intravascular coagulation, especially with M3 subtype.)

37-yo woman presents with 3 days of fever, fatigue, and rash. Her medications include oral contraceptives. Her temperature is 38.7°C; she also has nonpalpable petechial rash and splenomegaly. Coagulation studies and fibrinogen levels are normal, but a complete blood cell count shows a hemoglobin level of 9.7 g/dL and platelet count of 135,000/mm³. A peripheral blood smear shows schistocytes. Which of the following is the most likely diagnosis? (A) Disseminated intravascular coagulation (B) HELLP syndrome (C) Henoch-Schönlein purpura (D) Idiopathic thrombocytopenic purpura (E) Thrombotic thrombocytopenic purpura

E. (Thrombotic thrombocytopenic purpura is a disorder of increased platelet aggregation leading to mucocutaneous bleeding (petechiae or purpura) and hemolysis. Most cases are idiopathic, but the condition is associated with certain medications such as cyclosporine and clopidogrel, oral contraceptive use, HIV infection, and autoimmune disease. The classic pentad is thrombocytopenia, microangiopathic hemolytic anemia, mental status changes, fever, and renal failure, although the last three may not be present. Diagnosis is confirmed with unexplained thrombocytopenia and microangiopathic hemolysis; peripheral smear shows schistocytes)

34-yo G2P2 woman presents because she noticed some scant bloody discharge from her left nipple several days ago. She does not recall experiencing recent trauma to her breast, although she recently stopped breast-feeding her youngest child. Her vital signs are normal and she does not have any palpable breast masses or breast tenderness. A small drop of bloody fluid is expressed from the left nipple. Select the most appropriate next step: (A) A course of dicloxacillin (B) Breast ultrasound (C) Chemotherapy with doxorubicin (D) CT of the chest (E) Excisional biopsy (F) Fine-needle aspiration biopsy (G) Follow-up visit in 6 months (H) Incision and drainage (I) Lumpectomy with lymph node dissection (J) Modified radical mastectomy (K) Radiation therapy (L) Simple mastectomy (M) Stereotactic core biopsy

E. (Unilateral serous or serosanguineous nipple discharge from a single duct is most commonly caused by an intraductal papilloma. Other less likely causes include breast cancer or mammillary dysplasia with ectasia. The diagnosis of intraductal papilloma is made upon excision of the affected duct, which is also a curative procedure and allows for histologic examination of the lesion to rule out carcinoma.)

This sign refers to ecchymosis in the flank that suggests retroperitoneal hemorrhage accompanying severe acute pancreatitis.

Grey-Turner's

It is the most common systemic vasculitis in children and is caused by immune complex deposition in vessel walls. The clinical presentation includes palpable purpura beginning on the extensor surfaces and buttocks, polyarthralgia of the lower extremity joints, colicky abdominal pain, and nephritis. Platelet count is normal, whereas serum IgA levels are usually increased.

Henoch-Schönlein purpura

Reed-Sternberg cells can be seen in _____, which typically presents as lymphadenopathy rather than an elevated WBC count.

Hodgkin's lymphoma

It is a congenital form of hypertrophic cardiomyopathy and a common cause of sudden death among young athletes. It results from impaired relaxation and on echocardiography shows decreased left ventricular chamber size.

Idiopathic hypertrophic subaortic stenosis

Very uncommon type of lung cancer that is associated with gynecomastia. It carries a slightly worse prognosis than the other lung cancers.

Large cell carcinoma

67-yo woman comes to an outpatient surgical clinic for further evaluation of her mammogram, which reveals microcalcifications and irregular density. On PE there are no palpable breast masses but lymphadenopathy is present. Select the most appropriate next step: (A) A course of dicloxacillin (B) Breast ultrasound (C) Chemotherapy with doxorubicin (D) CT of the chest (E) Excisional biopsy (F) Fine-needle aspiration biopsy (G) Follow-up visit in 6 months (H) Incision and drainage (I) Lumpectomy with lymph node dissection (J) Modified radical mastectomy (K) Radiation therapy (L) Simple mastectomy (M) Stereotactic core biopsy

M. (This patient's physical exam findings - axillary lymphadenopathy and mammogram- microcalcifications and irregular density, are highly suspicious for ductal carcinoma in situ. Before any therapeutic options are discussed, a definitive tissue biopsy must be obtained. Stereotactic core needle biopsy is the preferred method in most centers, especially for nonpalpable lesions with microcalcifications on mammography)

This sign refers to the inspiratory arrest during deep palpation of the right upper quadrant in patients with acute cholecystitis.

Murphy's

Due to in vitro hemolysis of RBCs, causing them to release their potassium-rich contents. It occurs most frequently when there is a long delay between specimen collection and analysis.

Pseudohyperkalemia

Typically cause the paraneoplastic syndrome of hypercalcemia via production of PTH-related peptide (PTHrP). This type of cancer carry a better prognosis than small cell.

Squamous cell carcinoma

What is a hallmark of hairy cell leukemia?

pancytopenia

It is a marker for liver cancer, which normally presents with jaundice, elevated liver enzymes, and abdominal fullness. It is most common in individuals with cirrhosis, either from hepatitis infection or alcohol use.

α-Fetoprotein