HemelLymph/Integ Final Lecturio

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C. Halt treatments and begin palliative care.

A 19-year-old woman is diagnosed with metastatic Ewing sarcoma. She has undergone multiple treatments without improvement. She decides to stop treatment and pursue only palliative care. She is of sound mind and has weighed the benefits and risks of this decision. The patient's mother objects and insists that treatments be continued. What should be done? A. Continue treatments until the patient undergoes a psychiatric evaluation. B. Follow the wishes of the patient's mother as she has the decision-making power regarding the patient. C. Halt treatments and begin palliative care. D. Try to seek additional experimental treatments that are promising. E. Continue treatment because, otherwise, the patient will die.

D. Inhibit peripheral conversion of androgens to estrogen Aromatase inhibitors, such as anastrozole and letrozole, are first-line therapy for estrogen receptor-positive and progesterone receptor-positive breast cancer in postmenopausal women.

A 65-year-old woman with breast cancer returns to the outpatient oncology clinic for follow up and to discuss treatment options. A few months ago, she noticed a lump during a breast self-exam, and the subsequent work-up was positive for breast cancer. A lumpectomy revealed invasive ductal carcinoma that was estrogen receptor-positive and progesterone receptor-positive with nodal metastases. The patient had her last menstrual period 10 years ago and has not had any spotting since that time. Family history reveals her mother also had breast cancer. The patient remembers her having chemotherapy but did not tolerate it well, so she requests a different treatment approach. Which of the following is the mechanism of action of the best treatment option for this patient? A. Antagonist for estrogen receptors in the breast B. Cell cycle arrest C. Antagonist for estrogen receptors in the hypothalamus D. Inhibit peripheral conversion of androgens to estrogen E. Estrogen receptor downregulation in the breast

F. Depth of invasion of atypical cells the most important prognostic factor of melanoma is the depth of invasion of the disease into normal tissue. This determines the risk of metastasis and complications due to melanoma. Some of the more dangerous forms of melanoma, such as nodular melanoma, may appear with either normal or only slightly irregular superficial lesions, while there is a concurrent extensive depth of invasion. This depth can only be determined by biopsy, not by simply examining a lesion.

43-year-old woman presents to your clinic for the evaluation of an abnormal skin lesion on her forearm. The patient is worried because her mother passed away from melanoma. You believe that the lesion warrants biopsy for further evaluation for possible melanoma. Your patient is concerned about her risk for malignant disease. What is the most important prognostic factor of melanoma? A. Diameter > 6 mm B. S-100 tumor marker present C. Evolution of lesion over time D. Genetic factors E. Age at presentation F. Depth of invasion of atypical cells G. Level of irregularity of the borders

D. Vitamin C deficiency Vitamin C deficiency causes scurvy, the clinical features of which are petechiae, ecchymoses, glossitis, and bleeding gums.

A 6-year-old boy presents with bleeding gums. His past medical history reveals a recent supracondylar fracture of the right humerus due to a fall while playing. On physical examination, petechiae are seen all over the patient's body. The patient's tongue is shown in the image. Which of the following is the most likely cause of this patient's condition? A. Child abuse B. Osteogenesis imperfecta C. Menkes disease D. Vitamin C deficiency E. Ehlers-Danlos syndrome

C. Clindamycin

A 13-year-old girl presents with a right infected ingrown toenail. On examination, the skin on the lateral side of the toe is red, warm, swollen, and severely tender to touch. When gentle pressure is applied, pus oozes out. The patient is on no other regular medications but is allergic to sulfa drugs. Culture and sensitivity analysis of the pus shows methicillin-resistant Staphylococcus aureus (MRSA). Which of the following antibiotics is most effective against this organism? A. Cefotetan B. Oral vancomycin C. Clindamycin D. Cefuroxime E. Aztreonam

E. Polycythemia vera Polycythemia vera is the most likely cause of this patient's portal vein thrombosis (PVT). As its name implies, PVT is characterized by an abrupt onset of portal venous occlusion caused by a thrombus. Acute PVT is either clinically silent or may demonstrate sudden abdominal pain or pain that progresses over a few days. Fever and dyspepsia may also be present. Abdominal distention and fluid waves may be found on the physical examination. Chronic liver disease stigmata may be found in patients who also have cirrhosis.

A 14-year-old boy is brought to the office by his parents because he states that he has been feeling constantly tired for the past two months. He also reports a dull pain in the pit of his stomach. The patient has no relevant family history. His pulse is 105/min, respirations are 16/min, temperature is 37.0°C (98.6°F), and blood pressure is 111/66 mm Hg. On physical examination, his abdomen is distended, with hepatomegaly 5 cm (2.0 in) underneath the xiphoid process. The complete blood count results are as follows: Hemoglobin17.6 g/dLHematocrit64%RBC6.02 x 1012/L Leukocyte count 26,300/mm3Neutrophils55%Bands2%Eosinophils1%Basophils0%Lymphocytes29%Monocytes2%Platelet count480,000/mm³Erythropoietin< 1.0 mU/mL The coagulation test results are as follows: Partial thromboplastin time (activated)30.9 sProthrombin time14.0 s The abdominal Doppler ultrasound image is shown in the picture. What is the most likely etiology of this patient's diagnosis? A. Behçet syndrome B. Antiphospholipid syndrome C. Protein C deficiency D. Factor V Leiden mutation E. Polycythemia vera

E. Hidradenitis suppurativa Hidradenitis suppurativa is a recurring dermatologic condition characterized by the formation of painful inflammatory nodules, sinus tracts with seropurulent discharge, abscesses, and bands of severe scar formation. It affects areas of the skin with abundant apocrine glands such as the axillae, groin, breasts, and buttocks. The diagnosis is based on clinical criteria with either: One or more active lesions in the axilla or groin with ≥ 3 past episodes of suppurative, painful lesions, OR Inactive disease with ≥ 5 past episodes of characteristic suppurative, painful lesions in the axilla or groin

A 21-year-old man presents to his physician because of a recurrent painful rash (shown in the image) over the past 6 months. The rash initially began as a few small bumps and now involves a larger area with occasional white discharge. He has had similar episodes in his right axilla and groin. He has no significant past medical history other than obesity and he does not take any regular medication. His vital signs are within normal limits. Physical exam reveals tender papules and pustules in the left axilla without lymphadenopathy. Which of the following is the most likely diagnosis? A. Blastomycosis B. Cat-scratch disease C. Granuloma inguinale D. Erysipelas E. Hidradenitis suppurativa

E. It inhibits dihydrofolate reductase.

A 25-year-old woman first presented to your clinic due to morning stiffness, symmetric arthralgia in her wrist joints, and fatigue. She had a blood pressure of 132/74 mm Hg and a heart rate of 84/min. Physical examination revealed tenderness to palpation of both wrists but full range of motion. Test results for anti-citrullinated protein antibodies were positive, and the erythrocyte sedimentation rate was above the normal range. She was started on methotrexate therapy. She returns for follow-up 2 months later and is found to have megaloblastic anemia. What is the mechanism of action of methotrexate? A. It elevates tetrahydrofolate levels. B. It elevates methylmalonic acid levels. C. It intercalates into strands of DNA. D. It inhibits vitamin B12 activation. E. It inhibits dihydrofolate reductase.

A.Corticosteroids

A 26-year-old woman presents with blood in her urine for the past 2 days. She says she has had increasing urinary frequency at night for the past several days and recently noticed a reddish tinge in her urine. She is also concerned that her feet are beginning to swell, and that she has been feeling increasingly fatigued for the past week. She has no history of joint pains, rashes, or skin changes. Her medical history is relevant only for an occasional bluish discoloration of her fingers during exposure to cold. Her vital signs are a pulse of 80/min, a respiratory rate of 14/min, and blood pressure of 140/88 mm Hg. On physical examination, the patient has 1+ pitting edema of her feet bilaterally. The remainder of the examination is unremarkable. Laboratory findings are significant for the following: Serum glucose (fasting)88 mg/dL Sodium143 mEq/L Potassium3.7 mEq/L Chloride102 mEq/L Serum creatinine1.7 mg/dL Blood urea nitrogen32 mg/dL Cholesterol, total180 mg/dL HDL-cholesterol43 mg/dL LDL-cholesterol75 mg/dL Triglycerides135 mg/dL Hemoglobin12.5 g/dL Mean corpuscular volume (MCV)80 fL Reticulocyte count1 %Erythrocyte count 5.1 million/mm3 Thyroid-stimulating hormone 4.5 mU/mL Urinalysis: Glucose negative Protein+++ Ketones negative Nitrites negative RBCs negative Casts+++A renal biopsy reveals findings consistent with lupus nephritis (LN). Which of the following is the next best step in the treatment of this patient? A.Corticosteroids B.Azathioprine C.Cyclosporine D.Mycophenolic acid E.Cyclophosphamide

E. Polymerase chain reaction (PCR) for HIV RNA HIV RNA or DNA PCR is the diagnostic test of choice for detecting HIV infections in neonates/infants born to HIV-positive mothers. It is a rapid test and needs only a small dried spot of blood for initial screening

A 28-year-old primigravida at an estimated 16 weeks' gestational age presents for prenatal care. Routine prenatal screening tests reveal a positive HIV antibody test. The patient is extremely concerned that she may transmit HIV to her baby and wants to have the baby tested as soon as possible after delivery. Which of the following would be the most appropriate diagnostic test to address this patient's concern? A. EIA for HIV antibodies B. Antigen assay for p24 C. Viral culture D. CD4+ T cell count E. Polymerase chain reaction (PCR) for HIV RNA

D. Anti-histamines The patient has urticaria, probably due to type I hypersensitivity to peanuts. Peanut allergy can develop in childhood or less often in adulthood. H1 antihistamines (e.g., diphenhydramine, cetirizine) are usually enough to treat uncomplicated cases of urticaria. If signs and symptoms persist, corticosteroids can be given. Since this patient has normal vital signs and no signs of respiratory distress, there is no indication for epinephrine injection at this time. If treatment with H1 antagonists fails, or if she begins to develop respiratory distress, epinephrine should be given.

A 28-year-old woman presents with a sudden onset of round, red, and edematous skin lesions all over her body. The lesions are not painful but are pruritic. She also complains of severe genital itching. The patient also reports that she ate peanut butter 15-20 minutes before the rash and itching started. She has no signs of respiratory distress. Her blood pressure is 118/76 mm Hg, heart rate is 78/min, and respiratory rate is 14/min. Physical examination reveals clear lung sounds bilaterally with good air exchange. What is the suggested treatment? A. Antibiotics B. No medication, just observation C. Corticosteroids D. Anti-histamines E. Acyclovir

E. Vitamin B6 (pyridoxine)

A 29-year-old woman presents to the emergency department due to severe symptoms of alcohol intoxication and a seizure. She is accompanied by her husband, who reports that she takes disulfiram. There is no personal or family history of epilepsy. She shows signs of confusion, irritability, and depression. On further evaluation, she is noted to have stomatitis, glossitis, and cheilosis. Lab results reveal microcytic anemia and a chest X-ray is unremarkable. Which vitamin deficiency is likely present in this patient accounting for her physical findings? A. Vitamin B9 (folic acid) B. Vitamin B12 (cobalamin) C. Vitamin B2 (riboflavin) D. Vitamin B3 (niacin) E. Vitamin B6 (pyridoxine)

C. Cerebral edema The patient has a Plasmodium falciparum infection, as evidenced by the ring-form trophozoites in the smear shown in the image. Approximately half of the global population is at risk for malaria. Malaria is an epidemic disease in Africa, Southeast Asia, South America, Haiti, the Dominican Republic, and the Middle East. Fevers and chills in a traveler or an immigrant returning from an endemic region should be promptly investigated for malaria. Malaria classically presents with paroxysms of fevers with chills and rigors. The malarial paroxysm coincides with the release of merozoites from ruptured erythrocytes. Headaches, jaundice, anemia, splenomegaly, and hepatomegaly are other signs and symptoms of malaria. Additionally, the unconjugated bilirubin level will be elevated as the red blood cells (RBCs) rupture during the maturation process of the parasite.Unlike other forms, P. falciparum causes microvascular complications due to obstruction. Infected RBCs adhere to endothelial cells. Ischemia classically develops in the brain, kidneys, lungs, and gastrointestinal (GI) tract and can be fatal if untreated. Fever spikes occur intermittently at an interval of 48 hours. Patients with cerebral edema can present with irritability, seizures, and coma. Other complications include acute respiratory distress syndrome, diarrhea, shock-like syndrome (Algid malaria), thrombocytopenia, hypoglycemia, and lactic acidosis. Renal insufficiency may result from volume depletion and vascular obstruction by parasitized erythrocytes.

A 30-year-old woman seeks evaluation at a clinic complaining of shaking, chills, fevers, and headaches for the last three days. She recently returned from a trip to India, where she was visiting her family. There is no history of loss of consciousness or respiratory distress. Her temperature is 38.9℃ (102.0℉), respirations are 19/min, blood pressure is 120/80 mm Hg, and pulse is 94/min (rapid and thready). On general examination, she is pale, and the sclera is jaundiced. Laboratory studies show: Hematocrit (Hct)30%Total bilirubin 2.6 mg/dLDirect bilirubin 0.3 mg/dL A peripheral smear is shown in the picture. What is the most severe complication of this condition? Picture shows Ringed RBC A. Heart block B. Facial paralysis C. Cerebral edema D. Aplastic crisis E. Rheumatoid arthritis

E. Postherpetic neuralgia The woman has herpes zoster (shingles), which is a reactivation of the varicella-zoster virus (VZV). Normally, the VZV lays dormant in the dorsal root ganglion (DRG) of spinal sensory nerves after the acute infection (chickenpox) resolves. Reactivation usually happens because of immunocompromise, either due to age, disease, or certain medications and manifests as painful vesicles in the dermatomal distribution of the DRG where it had been residing.

A 31-year-old woman presents with painful vesicles on the right side of her torso. She notes that the lesions appeared two days ago and have not improved. One day prior to their appearance, she says that she experienced a burning sensation in the affected area. The patient is afebrile and vital signs are within normal limits. Upon physical examination, there are vesicles noted that are localized to the right T10 skin dermatome. Which of the following complications is associated with this woman's likely diagnosis? A. Fever B. Bacterial superinfection of the affected skin C. Pneumonia D. Cerebellar ataxia E. Postherpetic neuralgia

D. G2 phase Bleomycin is an antineoplastic agent that acts mostly during the G2 phase of the cell cycle. The mechanism of action is complex and not fully understood. It is a small peptide that contains a DNA-binding region at one end and an iron-binding domain at the opposite end. It acts by binding to DNA, resulting in single- and double-strand breaks following free radical formation, with consequent inhibition of DNA synthesis and accumulation of cells in the G2 phase of the cell cycle. The fragmentation of DNA is due to oxidation of a DNA-bleomycin-Fe(II) complex which leads to chromosomal aberrations (so it will also interfere with the mitotic phase, too).

A 33-year-old man with recently diagnosed testicular cancer visits his oncologist to discuss his treatment plan. His left testicle was removed after a thorough workup. A computed tomography scan of the pelvis showed no enlarged lymph nodes, and a simple orchiectomy and pelvic lymph node dissection were completed. The final diagnosis was stage IB (pT2 pN0 M0 S0) non-seminomatous testicular cancer. A combination of chemotherapeutic medications is recommended, including bleomycin, etoposide, and cisplatin. Each of the antineoplastic drugs has a different mechanism of action; each drug targets cancer cells at a specific phase in the cell cycle and works by inhibiting a major cellular process. Which part of the cell cycle does bleomycin affect the most? A. G0 phase B. G1 phase C. Synthesis phase D. G2 phase E. Mitotic phase

E. Sargramostim Sargramostim (granulocyte-macrophage colony-stimulating factor) is a hematopoietic growth factor or colony-stimulating factor used to improve the myelosuppression during HIV treatment, during chemotherapy, following a bone marrow graft, and after a stem cell transplant.

A 33-year-old woman presents to the clinic to follow up her AIDS treatment with highly active antiretroviral therapy (HAART). She is in good spirits and has been fully compliant with her medications and clinic visits. Other medications include prophylactic trimethoprim-sulfamethoxazole. Recent blood studies show the following: Hemoglobin11 g/dL Mean corpuscular volume 80 fL Reticulocyte count 0.5% Erythrocyte count 2 × 106/mm3 Leucocyte count 700/mm3 Neutrophils 40% Platelet count 20,000/mm3 Assays also show decreased viral DNA, decreased mRNA burden, and a significantly diminished viral load. To avoid modifying the effective antiretroviral therapy, she is started on a "blood-improving" medication. Thirty minutes after the first dose, the patient develops difficulty breathing, severe muscle pain, and vomiting. Her pulse is 120/min, and blood pressure is 80/50 mm Hg. Which of the following medications was most likely administered to the patient? A. Erythropoietin B. Thrombopoietin C. Interleukin 3 D. Platelet-derived growth factor E. Sargramostim

E. Human T-lymphotropic virus 1 (HTLV-1) This patient presents with chronic fatigue, lymphadenopathy, elevated serum calcium, and history of unprotected sex with multiple partners. The most likely diagnosis is adult T cell leukemia secondary to human T-lymphotropic virus 1 (HTLV-1). HTLV-1 is an RNA retrovirus that selectively infects T cells. Approximately 2%-5% of infected individuals will go on to develop adult T cell leukemia through a poorly understood process. HTLV-1 is a blood-borne infection transmitted sexually or through needle sharing and is endemic in Japan, Southeast Asia, and the Caribbean.

A 34-year-old man presents to an outpatient clinic with chronic fatigue and lumps in his neck, right axilla, and groin. He reports that he frequently visits Japan on business, and has had unprotected sex with multiple partners on these trips. On examination, generalized lymphadenopathy is noted. Laboratory testing reveals abnormal lymphocytes with morphologic atypia, condensed chromatin, and bizarre hyperlobated nuclei on peripheral smear. Serum calcium is 12.2 mg/dL. Which of the following viruses is associated with this patient's condition? A. Human immunodeficiency virus (HIV) B. Hepatitis C virus C. Hepatitis B virus D. Human T-lymphotropic virus 2 (HTLV-2) E. Human T-lymphotropic virus 1 (HTLV-1)

C. Tropical sprue This patient's signs and symptoms suggest folate deficiency anemia. Tropical sprue affects the jejunum and ileum, causing a malabsorption syndrome resulting in multiple nutritional deficiencies, including folate deficiency. Tropical sprue is caused by an unknown organism and typically occurs in tropical regions such as the Caribbean. Malabsorption occurs due to damage to the intestinal villi of the small bowel.

A 34-year-old man presents with chronic diarrhea that started gradually 3 months ago and has not improved. He denies any change in diet, but he recently returned from a long work trip to India. His medical history is otherwise unremarkable, and he is on no medications. On physical examination, the patient looks pale. His tongue is swollen and has a beefy, red appearance. Angular cheilitis is noted. Laboratory findings are significant for macrocytic, megaloblastic anemia, decreased serum folate, increased serum homocysteine, and normal methylmalonic acid levels. Tissue transglutaminase IgA antibodies are negative. Which of the following conditions is most likely causing this patient's symptoms? A. Autoimmune destruction of parietal cells B. Diphyllobothrium latum infection C. Tropical sprue D. Celiac disease E. Poor diet

E. Localized painless lymphadenopathy Localized painless lymphadenopathy is suspicious for a localized malignancy. Given the diagnostic work-up and referral to an oncologist, the clinician suspects thyroid cancer in this patient. In general, lymph node involvement by a malignancy often indicates a decreased survival rate and must be acted on expeditiously by physicians. Locally metastatic papillary thyroid cancer has a higher 5-year survival rate (99%) than most other cancers of the same stage.

A 34-year-old woman presents to her primary care physician for a routine check-up. She complains that she is not feeling like her usual self but has no specific complaints. Her physical examination reveals several significantly enlarged lymph nodes. The physician orders lab testing for thyroid-stimulating hormone (TSH), triiodothyronine (T3), and free thyroxine (T4). He also referred her to an oncologist for an initial consultation. Which type of lymphadenopathy was most likely present during the physical examination that made the primary care physician make this referral? A. Generalized painful lymphadenopathy B. Generalized painless lymphadenopathy C. Reactive lymphadenitis D. Localized painful lymphadenopathy E. Localized painless lymphadenopathy

B. Nodular lymphocyte-predominant Hodgkin lymphoma According to the histopathology and flow cytometry results, this patient most likely has nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL). This Hodgkin lymphoma (HL) variant occurs most commonly in men aged 30-40 years or in children. It differs from classic HL in that CD15, and CD30 Reed-Sternberg cells are absent, while CD19 and CD20-positive lymphocytes and histiocytes are abundant and resemble an exploded kernel of popcorn. Treatment for NLPHL consists of chemotherapy and/or radiation, and the prognosis is good.

A 35-year-old man presents with a mass on the central part of his neck. He reports it has been growing steadily for the past 2 weeks and is associated with fatigue and recurrent fevers. He has no significant past medical history. The patient denies any smoking, alcohol, or recreational drug use. He has not traveled in the past 6 months. On physical exam, there are multiple enlarged submandibular and cervical lymph nodes that are firm, mobile, and non-tender. A biopsy of one of the lymph nodes is performed and predominantly shows lymphocytes and histiocytes in a pattern "resembling popcorn." A flow cytometry analysis demonstrates cells that are CD19 and CD20-positive and CD15 and CD30-negative. What is the most likely diagnosis in this patient? A. Lymphocyte-rich classic Hodgkin lymphoma B. Nodular lymphocyte-predominant Hodgkin lymphoma C. Nodular sclerosis classic Hodgkin lymphoma D. Mixed cellularity classic Hodgkin lymphoma E. Lymphocyte-depleted Hodgkin lymphoma

B. Diffuse large B-cell lymphoma (DLBCL) Diffuse large B-cell lymphoma (DLBCL) is the most common high-grade B cell lymphoma associated with HIV infection. Other B cell non-Hodgkin lymphomas are follicular, Burkitt, mantle cell, marginal zone lymphomas, and lymphoplasmacytic lymphoma (also called Waldenstrom macroglobulinemia). HIV is also a risk factor for Burkitt lymphoma, but DLBCL is more common, especially in adults.

A 37-year-old man is being evaluated for fatigue that started about 3 weeks ago. He has a history of HIV infection diagnosed 7 years ago and is compliant with an antiretroviral regimen. Today, his CD4+ count is 350 cells/mm3. His partner passed away from a hematologic malignancy, and he is worried that his fatigue might be connected to a similar pathology. The physician explains that there is an increased risk of certain kinds of lymphoma in HIV-positive patients. Which one of the following conditions is the patient most likely to develop? A. Burkitt lymphoma B. Diffuse large B-cell lymphoma (DLBCL) C. Follicular lymphoma D. Small lymphocytic lymphoma E. Extranodal marginal zone lymphoma

B. Hemidesmosomes

A 37-year-old man presents to the clinic because of multiple itching blisters on his buttocks for the past week. One year ago, he noticed a similar outbreak on his inner thighs, but it receded spontaneously within a few days. Physical exam reveals blisters that are tense and do not rupture with rubbing or pressure. A biopsy demonstrates epidermal detachment from the basal lamina with subepidermal blisters containing extensive inflammatory infiltrates abundant with eosinophils. Immunofluorescence shows a linear pattern of immune complex deposits at the basement membrane. Which of the following cellular structures is most likely involved in the formation of these blisters? A. Gap junctions B. Hemidesmosomes C. Lamellar bodies D. Macula adherens E. Zonula occludens

E. Erythema nodosum an inflammatory process of subcutaneous fat, is associated with several conditions, including streptococcal pharyngitis, which this woman has. EN is characterized by elevated tender, erythematous, and immobile nodules distributed symmetrically on the anterior shins. The nodules do not ulcerate. EN can be associated with a variety of conditions, including ulcerative colitis, pregnancy, sarcoidosis, and systemic fungal infections. The diagnosis is typically based on patient history and clinical findings; however, the diagnosis can be confirmed with a skin biopsy, which predominantly shows septal panniculitis without primary vasculitis.

A 39-year-old woman presents to your office with four days of fever, sore throat, generalized aching, arthralgias, and tender nodules on both of her shins that arose in the last 48 hours. She denies having any chronic medical problems and she does not take any medications regularly. Temperature is 38.5°C (101.3°F), pulse is 85/min, and blood pressure is 120/65 mm Hg. She has tender and enlarged submandibular lymph nodes on physical exam, and enlarged erythematous tonsils with a patchy white exudate. You also note erythematous, tender, immobile nodules on both shins. What is the most likely diagnosis of the woman's cutaneous findings? A. Alpha-1 antitrypsin deficiency B. Erythema induratum C. Cutaneous polyarteritis nodosa D. Henoch-Schönlein purpura E. Erythema nodosum

C. Cutaneous lupus erythematosus (CLE) Cutaneous lupus erythematosus (CLE) is a subtype of systemic lupus erythematosus (SLE), an autoimmune disease. Symptoms associated with CLE are largely cutaneous and lack the systemic symptoms classically seen in SLE, although SLE can be accompanied by these cutaneous symptoms. There are two rashes most commonly associated with CLE: a malar (butterfly) rash, erythema across the face and bridge of the nose that spares the nasolabial folds, and a discoid rash, which is are scaly plaques on the scalp, neck, and ears. Both CLE and SLE are associated with antinuclear antibodies. Additionally, cutaneous lupus is often associated with anti-SSA antibodies. This woman's presentation and lab findings are consistent with discoid lupus erythematosus, a cutaneous form of lupus

A 42-year-old woman presents to the clinic for a recurrent rash that has remitted and relapsed over the last two years. She states that she has tried multiple home remedies when she has flare-ups but to no avail. She states that she is wary of medical care and has not seen a doctor in at least 15 years. On examination, she has multiple disc-shaped, scaly, erythematous lesions on her neck, progressing into her hairline. She denies any other symptoms. Lab work is performed and is positive for antinuclear antibodies. What is the most likely diagnosis? A. Drug-induced lupus B. Systemic lupus erythematosus (SLE) C. Cutaneous lupus erythematosus (CLE) D. Dermatomyositis E. Tinea capitis

D. A genetic test followed by colonoscopy for the son should be ordered. Hereditary non-polyposis colorectal cancer (HNPCC), or Lynch syndrome, is the most common inherited colorectal cancer. HNPCC is inherited with an autosomal dominant pattern, and the mutation causes defective mismatch repair. Patients with this mutation have a predisposition for carcinoma at an earlier age than does the general population and are at an increased risk for other cancers as well, including endometrial (30%-40% lifetime risk), small intestine, stomach, ovary, hepatobiliary, pancreas, upper urinary tract, brain, prostate, and skin. There are two types of Lynch syndrome. Lynch syndrome I is associated with familial colon cancer. Lynch syndrome II is associated with other gastrointestinal and reproductive tract cancers. These patients develop adenomas at the same rate as the general population, but progression to carcinoma is more likely. The average age of diagnosis of polyps is between the late 20s and early 30s; for colon cancer, it is 44 years (average age of diagnosis in the general population is 60-65 years).

A 45-year-old man presents with increasing fatigue and weakness for the past 3 months. He works as a lawyer and attributes his fatigue to stress at work lately. He has lost 2.3 kg (5 lb) during this time despite no changes in diet or activity level. His past medical history is significant for chronic constipation and infrequent episodes of bloody stools. Family history is significant for his father and paternal uncle, who both died of colon cancer and had a genetic mutation for the disease. The patient has never had a colonoscopy or any genetic testing performed. Physical examination is significant for conjunctival pallor. A colonoscopy is performed and reveals a few adenomatous polyps. Histopathologic examination shows high-grade dysplasia, and genetic testing reveals the same mutation as his father and uncle. The patient is concerned about his 20-year-old son. Which of the following is the most appropriate advice regarding this patient's son? A. The son should undergo a prophylactic colonic resection. B. An immediate colonoscopy should be ordered for the son. C. The son does not need to be tested at this time. D. A genetic test followed by colonoscopy for the son should be ordered. E. Screening for the son should start at age 45 years, as his risk for colon cancer is similar to that of the general population.

D. Dermatomyositis Dr. Elias-> Related to Cancer Women-> Breast and Ovarian Men-> Gastric and lymphoma

A 45-year-old woman presents to the clinic complaining of weakness that has progressively worsened over the past two weeks. She states that she has a hard time lifting both her arms, but they otherwise function normally. She notes no history of trauma or other deficits. On examination, she has 2/5 muscle strength on shoulder shrug and arm abduction bilaterally, but the neurological exam is otherwise normal. You notice some skin changes and ask her about them. She states that she has had a rash around her eyes and on her lower face, going down to her neck and chest. The rashes started around the same time as the weakness began. A complete blood count and basic metabolic panel are normal. Which of the following is the most likely diagnosis? A. Myasthenia gravis B. Polymyalgia rheumatica C. Lambert-Eaton myasthenic syndrome (LEMS) D. Dermatomyositis E. Fibromyalgia Next

B. Follicular hyperplasia

A 47-year-old woman presents to her physician for difficulty swallowing. She states that she intentionally delayed seeing a physician for this issue. She says her primary issue with swallowing is that her mouth always feels dry so she has difficulty chewing food to the point that it can be swallowed. On physical examination, her oral mucosa appears dry. Both of her eyes also appear dry. Several enlarged lymph nodes are palpated. Which of the following patterns of reactive lymphadenitis is most commonly associated with this patient's presentation? A. Sinus hyperplasia B. Follicular hyperplasia C. Paracortical hyperplasia D. Diffuse hyperplasia E. Mixed B and T cell hyperplasia

A. Acute lymphoblastic leukemia If the bone marrow blast percentage is greater than or equal to 25%, the diagnosis of leukemia is given. The presence of 35% blasts in the peripheral blood smear confirms the diagnosis of acute leukemia in this patient. Of all the childhood malignancies, acute leukemia is the most common, and acute lymphoblastic leukemia (ALL) makes up 75% of all leukemias in this age group. A bone marrow biopsy and flow cytometry should be done to confirm the diagnosis. Hepatosplenomegaly and a mediastinal mass are very common in leukemia (especially in cases of T-cell ALL).

A 5-year-old girl presents for a routine checkup. The parents say that she has been looking pale and tired lately. Her family history is unremarkable. Physical examination reveals several bruises and petechial bleeding on her limbs. Complete blood count demonstrates leukocytosis with severe anemia and thrombocytopenia. The peripheral blood smear shows 35% blasts. Ultrasonography of the abdomen shows hepatosplenomegaly, and a chest radiograph reveals a mediastinal mass. Which of the following is the most likely diagnosis? A. Acute lymphoblastic leukemia B. Acute myeloid leukemia C. Chronic lymphocytic leukemia D. Aplastic anemia E. Chronic myeloid leukemia

E. Lymphocytic infiltration of graft vessels and endothelial damage

A 50-year-old man presents to a clinic with oliguria. Four weeks ago, he had a kidney transplant. Postoperative follow-up was normal. He is currently on cyclosporine and admits that sometimes he forgets to take his medication. On physical examination, the vital signs include: temperature 37.1°C (98.8°F), blood pressure 165/110 mm Hg, heart rate 80/min, and respiratory rate 16/min. There is mild tenderness on renal palpation. His serum creatinine level is 4 mg/dL, well above his baseline level after the transplant. Which of the following best describes the histological finding if a biopsy is taken from the transplanted kidney? A. Thickening of blood vessels, fibrosis of graft vessels, and parenchymal atrophy B. Necrosis with granulation tissue C. Atherosclerosis on angiography D. Thrombosis and occlusion of vessels E. Lymphocytic infiltration of graft vessels and endothelial damage

C. Methotrexate This patient's clinical features and laboratory results are highly suggestive of rheumatoid arthritis-a chronic, systemic autoimmune disorder that primarily affects the weight-bearing joints and metacarpal joints in affected individuals. Patients are usually diagnosed by the clinical presentation of at least 6 weeks duration of morning stiffness that lasts more than 1 hour and worsens with rest, symmetric distribution of joint pain, and small joint synovitis in the hands that spares the distal interphalangeal joints. Important positive laboratory findings include rheumatoid factor and anti-cyclic citrullinated peptide (anti-CCP) antibodies. X-ray findings of the joints including periarticular osteopenia and marginal bony erosions are also important. Disease-modifying antirheumatic drugs (DMARDs) and biologic agents are used in the treatment of rheumatoid arthritis to limit disease activity and halt progression.

A 50-year-old woman presents to the clinic with joint pain that has persisted for the last 2 months. She reports having intermittently swollen, painful hands bilaterally. She adds that when she wakes up in the morning, her hands are stiff and do not loosen up until an hour later. The pain tends to improve with movement. Physical examination is significant for warm, swollen, tender proximal interphalangeal joints, metacarpophalangeal joints, and wrists bilaterally. Laboratory results are positive for rheumatoid factor (4-fold greater than the upper limit of normal (ULN)) and anti-cyclic citrullinated peptide (anti-CCP) antibodies (3-fold greater than ULN). CRP and ESR are elevated. Plain X-rays of the hand joints show periarticular osteopenia and bony erosions. She was started on the first-line drug for her condition which inhibits dihydrofolate reductase. Which medication was this patient started on? A. 5-fluorouracil B. Leflunomide C. Methotrexate D. Hydroxyurea E. Allopurinol

E. Sentinel node - right lumbar trunk - cisterna chyli - thoracic duct - left subclavian vein - systemic circulation

A 51-year-old Asian woman presents to her primary care physician with abdominal pain. Physical exam reveals rebound tenderness in the lower right quadrant. Further workup suggests right-sided ovarian cancer, which is confirmed later in the week to be stage 4 with metastases in the lungs and brain. Which of the following describes the most likely lymphatic route for this malignancy to have metastasized to the brain? A. Sentinel node - left lumbar trunk - cisterna chyli - right bronchomediastinal trunk - right lymphatic duct - right subclavian vein - systemic circulation B. Sentinel node - left lumbar trunk - cisterna chyli - thoracic duct - left subclavian vein - systemic circulation C. Sentinel node - right lumbar trunk - cisterna chyli - right bronchomediastinal trunk - right lymphatic duct - right subclavian vein - systemic circulation D. Sentinel node - right lumbar trunk - cisterna chyli - thoracic duct - right subclavian vein - systemic circulation E. Sentinel node - right lumbar trunk - cisterna chyli - thoracic duct - left subclavian vein - systemic circulation

A. Pemphigus vulgaris

A 52-year-old woman presents with erosions in her mouth that are persistent and painful. She says that symptoms appeared gradually 1 week ago and have gotten progressively worse. She also notes that, several days ago, flaccid blisters appeared on her skin, which almost immediately transformed into erosions as well. Which of the following is the most likely diagnosis? A. Pemphigus vulgaris B. Psoriasis C. Bullous pemphigoid D. Molluscum contagiosum E. Staphylococcal infection (scalded skin syndrome)

D. Metaplasia of the esophageal mucosa This patient's long-standing history of gastroesophageal reflux disease (GERD) has most likely resulted in Barrett's esophagus, which is metaplasia of the lower esophageal mucosa. In patients with Barrett's esophagus, the normal non-keratinized stratified squamous epithelium is replaced by non-ciliated columnar epithelium with goblet cells. This metaplasia is a reaction to the damage caused by the backflow of gastric acid, which leads the stem cells in the esophagus to produce a protective epithelium. Barrett's esophagus is a risk factor for developing adenocarcinoma of the esophagus.

A 54-year-old man with worsening refractory epigastric pain secondary to long-standing gastroesophageal reflux disease (GERD) presents for endoscopic evaluation. His medical history is significant for type 2 diabetes mellitus, diagnosed 3 years ago. Current medications are metformin and omeprazole. Which of the following best describes this patient's most likely endoscopic findings? A. Longitudinal lacerations of the esophageal mucosa B. Esophageal smooth muscle atrophy C. Hypertrophy of the esophageal mucosa protruding into the lumen of the lower esophagus D. Metaplasia of the esophageal mucosa E. A malignant proliferation of squamous cells

C. t(15;17) This patient has clinical features and laboratory findings highly suggestive of acute myelogenous leukemia (AML), specifically acute promyelocytic leukemia (APL, formerly called M3), with Auer rods noted in his peripheral blood smear. AML is a hematologic malignancy characterized by uncontrolled proliferation of myeloid precursor cells. It is predominantly seen in older adults with a median age at diagnosis approximately 65 years, and the incidence increases with age, but APL is seen more often in adults under age 60. Acute promyelocytic leukemia is associated with the translocation t(15;17).

A 55-year-old man presents with complaints of fatigue, weakness, and bleeding gums for the past 3 weeks. He also says he cut his finger while cooking, and the bleeding took more than 10 minutes to stop. He has a family history of type 2 diabetes mellitus and prostate cancer. He takes no daily medications other than a multivitamin supplement. His temperature is 36.7°C (98.0°F), heart rate is 78/min, respiratory rate is 14/min, and blood pressure is 122/67 mm Hg. On physical examination, the patient seems pale and lethargic. Hepatosplenomegaly is noted. A peripheral blood smear reveals myeloblasts positive for myeloperoxidase and Auer rods. A bone marrow biopsy shows > 30% of blast cells. Which of the following chromosomal abnormalities is associated with this patient's most likely diagnosis? A. JAK2 mutation B. t(9;22) C. t(15;17) D. t(11;14) E. t(8;14)

A. Lymphocyte-depleted Hodgkin lymphoma Lymphocyte-depleted Hodgkin lymphoma (HL) represents 2% and has the worst prognosis of all HLs. Like other HLs, histology shows multinucleated cells with an "owl-eye" appearance, representing the classic Reed-Sternberg (RS) cells. Lymphocyte-depleted HL is associated with HIV disease. Histologically, this variant is characterized by diffuse fibrosis with abundant RS cells with bizarre features and a hypocellular background. Treatment consists of chemotherapy.

A 55-year-old man presents with severe fatigue and fever. His medical history is significant for a new diagnosis of HIV disease 8 weeks ago. At that time, he was also started on antiretroviral therapy. On physical examination today, the patient is pale. A chest radiograph shows multiple enlarged mediastinal lymph nodes. A biopsy of one of the enlarged nodes shows multinucleated cells with an "owl-eye" appearance with a hypocellular background. This patient's condition is very aggressive and has a poor prognosis. What is the most likely diagnosis in this patient? A. Lymphocyte-depleted Hodgkin lymphoma B. Diffuse large B cell lymphoma C. Follicular lymphoma D. Extranodal marginal zone lymphoma E. Nodular lymphocyte-predominant lymphoma

C. Mixed cellularity classic Hodgkin lymphoma Based on the histological picture, this patient most likely has mixed cellularity classical Hodgkin lymphoma (MCCHL). Hodgkin lymphoma (HL) is a B cell malignancy with many variants. The MCCHL variant of HL classically occurs in men aged > 55 years and in patients who are HIV-positive. Additionally, HL has an association with EBV infection, an oncogenic virus. In addition to "B symptoms" of fever, night sweats, and weight loss, patients often present with painless lymphadenopathy. Histologically, classic CD15 and CD30-positive Reed-Sternberg (RS) cells are seen in the background with eosinophils, plasma cells, and histiocytes. Treatment is with chemotherapy and/or radiation, and the prognosis is good.

A 58-year-old man presents with a lump on his neck. He says he first noticed the mass 2 months ago, and it has been progressively enlarging. He denies any pain, weight loss, fevers, chills, or night sweats. His medical history is significant for HIV diagnosed 5 years ago and managed with a new antiretroviral treatment regimen that he just started. The patient is afebrile, and his vital signs are within normal limits. Physical exam reveals a 3 cm (1.2 in) mobile firm mass on the left lateral neck immediately below the level of the thyroid cartilage. A biopsy of the mass is performed and reveals atypical mononuclear cells in a background of eosinophils, plasma cells, histiocytes, atypical T lymphocytes, and bilobed cells (shown in the image). Which of the following is the most likely diagnosis in this patient? A. Lymphocyte-rich classic Hodgkin lymphoma B. Nodular lymphocyte-predominant Hodgkin lymphoma C. Mixed cellularity classic Hodgkin lymphoma D. Nodular sclerosis classic Hodgkin lymphoma E. Lymphocyte-depleted Hodgkin lymphoma

E. Necrotizing fasciitis This patient has two risk factors for developing necrotizing fasciitis--diabetes mellitus and chronic alcohol abuse.

A 58-year-old woman with a history of chronic alcohol abuse presents to the emergency department for complaints of leg pain and the skin changes seen in the image. She had an intramuscular injection in the same leg 2 days ago, and her symptoms began last night. She has had diabetes mellitus for 13 years. Her medications include aspirin, metformin, and insulin. She appears confused. Blood pressure is 90/70 mm Hg, pulse is 115/min, respiratory rate is 21/min, and temperature is 39.5℃ (103.1℉). Serum creatinine is 2.5 mg/dL. Which of the following is the most likely diagnosis? A. Cellulitis B. Ecthyma gangrenosum C. Erysipelas D. Fournier gangrene E. Necrotizing fasciitis F. Pyoderma gangrenosum G. Toxic epidermal necrolysis

C. Recent immigration from equatorial Africa

A 6-year-old boy is brought to a primary care provider by his adoptive parents for evaluation of a 3-month history of jaw swelling. He has a travel history of recent immigration from equatorial Africa, where his deceased mother was positive for HIV and died from related complications. On physical exam, extensive lymph node swelling on the left side of his jaw is noted. There is also an ulcer that appears to be infected. A fine-needle biopsy of the lymph node yields a diagnosis of Burkitt lymphoma by the pathologist. Which of the following put this patient at the highest risk for this diagnosis? A. Infected ulcer B. Close family member with HIV C. Recent immigration from equatorial Africa D. Gender of the patient E. Ethnicity of the patient

C. The blood count will contain band forms, metamyelocytes, and myelocytes. The patient most likely has a leukemoid reaction, which must be differentiated from leukemia. Leukemoid reaction can occur secondary to severe or chronic infection, metastatic malignancies, or severe hemolysis. It presents with a high leukocyte count > 50 × 109/L and a marked neutrophil left shift with an elevated LAP score.

A 6-year-old boy presents to the emergency department with signs of sepsis. Laboratory studies reveal a leukocytosis of 60 × 109/L with marked left shift, but blast cells are absent. The patient is febrile and dehydrated. The physician believes that the patient has a severe reaction to the infection and orders a leukocyte alkaline phosphatase (LAP) stain on a peripheral smear. The LAP score is elevated. Which of the following statements is accurate regarding this child's condition? A. This condition can lead to chronic myelocytic leukemia. B. Myeloblasts and promyelocytes are expected to be found. C. The blood count will contain band forms, metamyelocytes, and myelocytes. D. Chemotherapy is the treatment of choice. E. The patient may develop anemia secondary to infection.

A. Chronic myeloid leukemia which is characteristically associated with the t(9;22)(q34;q11) translocation, the Philadelphia chromosome. This translocation results in the formation of the BCR-ABL1 fusion gene, due to translocation of the ABL1 gene (located on chromosome 9) next to the BCR gene (major breakpoint cluster region gene) on chromosome 22. ABL1 gene is a cellular oncogene. The BCR-ABL1 gene encodes for p210BCR-ABL1, which is a novel oncoprotein that exhibits constitutive tyrosine kinase activity, resulting in the excessive proliferation and decreased apoptosis of CML cells. CML is a common myeloproliferative disorder, accounting for 15-20% of leukemias. It has a slight male preponderance and usually presents at 55-65 years of age. It is characterized by the abnormal proliferation of the granulocytic cell line, although the capacity to differentiate is retained. Clinical presentation of CML is classified into 3 phases: chronic, accelerated, and blast. The chronic phase may be detected incidentally on a routine medical examination or may present with clinical features such as fatigue, decreased exercise tolerance, weight loss, decreased appetite, fever, excessive perspiration, unexplained leukocytosis, splenomegaly, or hepatomegaly. Complete blood counts usually suggest anemia, leukocytosis, and thrombocytosis; the presence of thrombocytopenia indicates a poorer prognosis. Total leukocyte count may be 20,000-60,000/mm3 at presentation with a significant increase in mature granulocytes and normal lymphocytes. Eosinophilia and/or basophilia may also be present. A peripheral blood smear shows a leukoerythroblastic blood picture

A 60-year-old man presents to his physician as part of his routine annual medical check-up. He has no specific complaints but mentions that he has often experienced fatigue over the past few months. His past medical history is noncontributory. On physical examination, his temperature is 37.2°C (98.8°F), pulse rate is 84/min, blood pressure is 130/86 mm Hg, and respiratory rate is 18/min. On general examination, mild pallor is present. Palpation of the abdomen reveals splenomegaly, which extends 6.35 cm (2.5 in) below the left costal margin. There is no hepatomegaly. Laboratory studies show the following values: Hemoglobin9.7 g/dL Total leukocyte count30,000/mm3 Granulocytes83% Lymphocytes10% Eosinophils5% Basophils1% Monocytes1% Platelet count700,000/mm3 The physician orders a bone marrow biopsy analysis of hematopoietic cells. The report shows the presence of a t(9;22)(q34; q11) translocation. Which of the following is the most likely diagnosis? A. Chronic myeloid leukemia B. Chronic myelomonocytic leukemia C. Chronic neutrophilic leukemia D. Transient myeloproliferative disorder E. Myelodysplastic syndrome/myeloproliferative neoplasm, unclassifiable

D. Tartrate-resistant acid phosphatase (TRAP) ANSWER Hairy cell leukemia exhibits tartrate-resistant acid phosphatase (TRAP) positivity. Hairy cell leukemia is a B cell neoplasm that typically affects men > 50 years of age. It is a very slow-growing leukemia for which treatments are often delayed or omitted entirely. Roughly 90% of patients will have an enlarged spleen.

A 61-year-old man presents to the emergency department with abdominal discomfort and malaise over the past 2 weeks. He states that he is married and monogamous. He has a temperature of 39.4°C (102.9°F) and complains of night sweats. On physical examination, the man has an enlarged spleen with mild tenderness and pale nail beds. There is mild tonsillar erythema, and a pulmonary examination demonstrates scattered crackles. A complete blood count demonstrates anemia, thrombocytopenia, and leukocytosis with lymphocytic predominance. A bone marrow aspiration is scheduled for the next morning based on the peripheral blood smear findings but is inconclusive due to a low yield. The patient is admitted to the hospital due to the anemia and given a transfusion of packed red blood cells and wide-spectrum antibiotics. He is released home the next day with instructions for primary care follow-up. Which of the following laboratory findings is most reliably positive for the primary cause of this illness? A. Rapid streptococcal antigen B. QuantiFERON-TB Gold C. Monospot D. Tartrate-resistant acid phosphatase (TRAP) E. CD 25

B. Multiple myeloma

A 61-year-old woman presents to her primary care physician for a routine check-up. Physical examination demonstrates asymmetric peripheral neuropathy in her feet. The patient has no previous relevant history and denies any symptoms of diabetes. Routine blood work shows normal results, and she is referred to a hematologist. Subsequent serum protein electrophoresis demonstrates a slightly elevated gamma globulin level, and monoclonal gammopathy of undetermined significance is diagnosed. Which of the following diseases is most likely to develop over the course of this patient's condition? A. Waldenström's macroglobulinemia B. Multiple myeloma C. Acute myelocytic leukemia D. Chronic lymphocytic leukemia E. Chronic myelocytic leukemia

B. Mutation of the JAK2 gene is commonly seen in this condition. Polycythemia vera (PV) is a myeloproliferative neoplasm causing excessive proliferation of the erythroid, myeloid, and megakaryocyte lineages. The disease may rarely progress to CML, AML, and myelofibrosis (Answer C). PV is commonly observed among refinery workers. Patients are at risk for thrombotic complications due to hyperviscosity of the blood, 1 of the 3 features of the Virchow's triad: endothelial injury, abnormal blood flow, and hypercoagulability. The factors may act independently or in combination to form a thrombus, even though endothelial injury is the most significant of them; hyperviscosity syndromes, leading to turbulent blood flow, can also promote thrombus formation.

A 62-year-old man, a retired oil pipeline engineer, presents to his primary care physician with complaints of headaches, fatigue, and constant ringing in his ears. Recurrently he has developed pruritus, usually after a hot shower. He also noted a constant burning sensation in his fingers and toes, independent of physical activity. On examination, he has a red face and his blood pressure levels are 147/89 mm Hg. A CBC revealed that his Hb is 19.0 g/dL and Hct is 59%. Because of his condition, his physician prescribes him 81 mg of aspirin to be taken daily in addition to therapeutic phlebotomy. Which of the statements below is true about this patient's condition? A. Arterial oxygen saturation is usually higher than normal values in this condition. B. Mutation of the JAK2 gene is commonly seen in this condition. C. The patient has a decreased risk of developing myelofibrosis. D. Serum erythropoietin is expected to be high. E. Warfarin and phlebotomy are the preferred course of treatment.

A. The tumor cells exhibit marked nuclear atypia. The presence or absence of marked nuclear atypia in tumor cells is important for the grading of a neoplasm. As the patient's physician notes, although it is possible to grade, though not stage, the neoplasm based on currently available diagnostic information, grading provides the physician with information about the degree of nuclear atypia exhibited by the tumor cells.

A 62-year-old woman presents to her physician with a painless breast mass on her left breast for the past 4 months. She mentions that she noticed the swelling suddenly one day and thought it would resolve by itself. Instead, it has been slowly increasing in size. On physical examination of the breasts, the physician notes a single non-tender, hard, and fixed nodule over left breast. An ultrasonogram of the breast shows a solid mass, and a fine-needle aspiration biopsy confirms the mass to be lobular carcinoma of the breast. When the patient asks about her prognosis, the physician says that the prognosis can be best determined after both grading and staging of the tumor. Based on the current diagnostic information, the physician says that they can only grade, but not stage, the neoplasm. Which of the following facts about the neoplasm is currently available to the physician? A. The tumor cells exhibit marked nuclear atypia. B. The tumor invades the pectoralis major. C. The tumor has metastasized to the axillary lymph nodes. D. The tumor has not metastasized to the contralateral superior mediastinal lymph nodes. E. The tumor has spread via blood-borne metastasis.

B. Kidney damage

A 68-year-old man presents to his primary care physician for a routine wellness exam. He currently has no complaints. He has a slightly elevated calcium level (10.7 mg/dL) on lab testing, and some plasma cells are found in his peripheral blood smear (less than 10%). His physician orders a serum protein electrophoresis which demonstrates a slight increase in gamma protein that is light chain predominate. What is the most likely complication of disease progression in this patient if left untreated? A. Peripheral neuropathy B. Kidney damage C. Raynaud phenomenon D. Splenomegaly E. Hepatomegaly

A. Direct immunofluorescence study

A 68-year-old man presents with blisters on the flexor surfaces of his arms and legs. He notes that the lesions appeared 2 days ago and have not improved. He says that he has had similar blisters in the past but has not sought medical attention until now. The man has no significant past medical history. He is afebrile and his vital signs are within normal limits. On physical examination, there are tense bullae present on the flexor surfaces of his arms and legs. Biopsy of a lesion and histopathologic examination reveals a subepidermal blister with a polymorphous but predominantly eosinophilic infiltrate. Which of the following is the best next diagnostic step in this patient? A. Direct immunofluorescence study B. Indirect immunofluorescence study C. Swab and culture of the blister D. Potassium hydroxide preparation (KOH prep) of the blister E. Bacteriological examination of fluid trained from the blister

D. Vincristine Vincristine is a category D drug and is contraindicated in pregnancy due to evidence of human fetal risk. In this clinical vignette of a patient with ALL, a cranial nerve palsy occurs during the course of her chemotherapy. Among the drugs listed, vincristine is known to cause such an adverse reaction. The peripheral neuropathy can be treated with pyridoxine, and recovery is complete in most cases. Of note, the medication is a vesicant and should be administered through central intravenous access.

A 7-year-old girl presents to the pediatrician with a low-grade fever, lethargy, and fatigue for the past week. The mother says that she has also complained of leg pain for the past two weeks. There is no significant past medical history. She was born at term via spontaneous vaginal delivery with no complications. Physical examination reveals generalized pallor and cervical lymphadenopathy. Bone marrow biopsy confirms the diagnosis of acute lymphoblastic leukemia (ALL). The patient is started on a chemotherapy regimen consisting of vincristine, daunorubicin, L-asparaginase, and prednisolone for induction, followed by intrathecal methotrexate for maintenance. Following the fourth cycle of chemotherapy, she develops bilateral ptosis. She is started on pyridoxine and pyridostigmine, and has a complete resolution of the ptosis within seven days. Which of the following drugs is most likely associated with this patient's initial adverse reaction? A. Daunorubicin B. Prednisolone C. Methotrexate D. Vincristine E. Pyridoxine

D. Cyclophosphamide Cyclophosphamide is an anticancer agent that is classified as an alkylating agent. It is used in the treatment of a number of malignancies, including non-Hodgkin lymphoma. One of the most common adverse effects of cyclophosphamide is hemorrhagic cystitis, which should be in the differential diagnosis of any patient with a history of recent chemotherapy who presents with hematuria.

A 70-year-old man presents to a medical clinic reporting blood in his urine and lower abdominal pain for the past few days. He is also concerned about urinary frequency and urgency. He states that he recently completed a cycle of chemotherapy for non-Hodgkin lymphoma. Which chemotherapy agent has most likely caused his symptoms? A. Cytarabine B. Methotrexate C. Rituximab D. Cyclophosphamide E. Prednisone

E. Essential thrombocythemia This patient has polycythemia vera (PV). PV is a neoplastic disorder with the dysregulation of myeloid cell development, especially RBCs, caused by a JAK2 mutation in chromosome 9. Other diseases associated with JAK2 mutation include essential thrombocythemia and chronic idiopathic myelofibrosis. Patients with PV classically present with dizziness, headaches, flushed red face, itching after bathing, transient visual disturbances, burning pain and redness in the feet or hands, and epigastric pain. These patients are at an increased risk for both thrombotic events and hemorrhage; they may present with deep vein thrombosis, pulmonary embolus, myocardial infarction, or bleeding.

A 70-year-old man presents to his primary care physician with complaints of dizziness, headaches, itching, and epigastric pain. Physical exam reveals hepatosplenomegaly and a flushed, red face. Lab results show elevated hemoglobin, hematocrit, and platelet count. Serum erythropoietin (EPO) is low. Genetic testing shows a JAK2 mutation. An upper GI endoscopy shows gastroduodenal erosions. Hypercellularity with fibrosis is seen on bone marrow aspirate. The patient is informed about treatment options for his condition, including hydroxyurea and ruxolitinib. Which of the following disorders can develop due to the same mutation as in this patient? A. Acute monocytic leukemia B. Mantle cell lymphoma (MCL) C. Chronic myelogenous leukemia (CML) D. Burkitt lymphoma E. Essential thrombocythemia

E. Sex Given the history of blood-tinged sputum, unintentional weight loss, CT scan findings, and biopsy findings, it is most likely that this patient has adenocarcinoma of the lung, the most frequently observed type of lung cancer in non-smoking women over age 60. Since this patient is a lifetime non-smoker and had no significant exposure to secondhand smoke, the only risk factor, in this case, is sex; adenocarcinoma is more prevalent in women than in men. A higher percentage of women with lung cancer are never smokers compared with men, (especially in Japan, although not related to this patient). Another risk factor for lung cancer is age. The risk of lung cancer increases with age in both smokers and non-smokers.

A 70-year-old white woman presents with a 2-week history of blood-tinged sputum. Her past medical history is significant for peptic ulcer disease, for which she took triple-drug therapy. She has never smoked and worked as a teacher before retiring at age 60. A review of systems is significant for a weight loss of 6.8 kg (15 lb) over the last 5 months. Her vitals include: blood pressure 135/85 mm Hg, temperature 37.7°C (99.9°F), pulse 95/min, and respiratory rate 18/min. Physical examination is unremarkable. A contrast CT scan of the chest shows an irregular mass in the peripheral region of the inferior lobe of the right lung. A CT-guided biopsy is performed and reveals malignant tissue architecture and gland formation with a significant amount of mucus. Which of the following is the most significant risk factor for this patient's most likely diagnosis? A. Medications B. Occupational history C. Past medical history D. Race E. Sex

D. Bone depletion due to chronic corticosteroid use

A 71-year-old woman presents to the clinic after an X-ray that revealed compression fractures of her L1 and L2 vertebral bodies due to osteoporotic changes. The patient has a history of hypertension for which she takes hydrochlorothiazide, and rheumatoid arthritis, for which she has been taking prednisone for the last 2 years. The patient states that she had a dual-energy X-ray absorptiometry (DEXA) scan 3 years ago that was normal and attributes that finding to regularly taking calcium and vitamin D supplements since menopause. The patient states that her pain from the fractures is stopping her from participating in her regular activities, such as exercising and gardening. Which of the following is the main cause of her osteoporosis? A. Decreased estrogen levels B. Hydrochlorothiazide (HCTZ) therapy C. Calcium malabsorption D. Bone depletion due to chronic corticosteroid use E. Undiagnosed hyperparathyroidism

C. Formation of interstrand DNA cross-links Cisplatin acts by forming interstrand and intrastrand cross-links which bind DNA, thus inhibiting DNA synthesis and function. The main binding site is the N7 position of guanine; the N3 position of adenine and the O6 position of cytosine are the other binding sites. Cisplatin also binds to cytoplasmic and nuclear proteins, and this also contributes to its antineoplastic activity (see image below). Cisplatin is a platinum analog that kills tumor cells in all phases of the cell cycle, with cells in the G1 and S phases being most vulnerable to the actions of platinum analogs.

A 72-year-old man has recently been diagnosed with stage 3 squamous cell carcinoma of the oral cavity. After the necessary laboratory workup, concurrent chemoradiation therapy is recommended. Radiotherapy is planned over 7 weeks and the patient is to receive radiation doses daily, Monday-Friday, in 2.0 Gy fractions. For concurrent chemotherapy, he will receive intravenous cisplatin at a dosage of 50 mg/m2 weekly for 7 weeks. Which of the following best explains the mechanism of action of the antineoplastic drug that the patient will receive? A. Inhibition of tubulin polymerization B. Inhibition of topoisomerase 2 C. Formation of interstrand DNA cross-links D. Inhibition of topoisomerase 1 E. Free radical-mediated lipid peroxidation

C. AL amyloid

A 72-year-old man is diagnosed with multiple myeloma. His blood work shows mild anemia, and a urinalysis detects proteinuria at a level of 670 mg/24 hr. A kidney biopsy is performed. Which of the following deposits would most likely be seen on the renal biopsy in this patient? A. Аβ amyloid B. Calcitonin C. AL amyloid D. Mutated transthyretin E. Amylin

B. Chronic lymphocytic leukemia Lymphocytosis that persists over several months with no clear etiology, especially in an older person, should raise the suspicion of chronic lymphocytic leukemia (CLL). CLL is diagnosed with more than 5000 lymphocytes/mm3 (e.g., 40% of 20,000 WBC in this patient) in the peripheral blood and more than 30% of monoclonal lymphocytes in the bone marrow. These cells will be positive for CD 5, 19, 20, and 23 and negative for terminal deoxytransferase (TdT). Symptoms include fatigue and lymphadenopathy. Many patients are asymptomatic, and the diagnosis is made on a CBC done for other reasons. As the disease progresses, anemia and thrombocytopenia can develop and, later, hepatosplenomegaly. Regular follow-up every 3 months is recommended. The treatment of CLL depends on the patient's age and the stage of the disease.

A 72-year-old man returns to his physician to follow up on abnormal lab results showing lymphocytosis 3 months ago. He has been feeling a bit more tired lately but does not complain of any other symptoms. His chronic medical problems include hypertension and hyperlipidemia. He takes lisinopril, hydrochlorothiazide, and atorvastatin. Surgical history is significant for right hip replacement 3 years ago for severe osteoarthritis. The family history is noncontributory. He drinks socially and does not smoke. Vital signs today are temperature 36.8°C (98.2°F), heart rate 94/min, respiratory rate 16/min, and blood pressure of 135/85 mm Hg. On physical examination, he appears well. His heart has a regular rate and rhythm and the lungs are clear to auscultation bilaterally. He has mild anterior cervical lymphadenopathy. A complete blood count and differential shows: WBC count20,000/mm3 RBC count3.1 million/mm3 Hemoglobin11.0 g/dL MCV95 μm3 MCH29 pg/cell Platelet count150,000/mm3 Neutrophils40% Lymphocytes40% Monocytes5% A specimen is sent for flow cytometry and shows antibodies specific for CD5, CD19, CD20, and CD23. What is the most likely diagnosis? A. Aplastic anemia B. Chronic lymphocytic leukemia C. Tuberculosis D. Acute lymphoblastic leukemia E. Immune thrombocytopenic purpura

D. Itraconazole The man has a fungal skin infection known as sporotrichosis (rose gardener's disease), and the first-line treatment is with itraconazole. It is caused by Sporothrix schenckii, a dimorphic fungus with a characteristic cigar-shaped, budding yeast. The pathogen is commonly found in soil and can cause disease once introduced into a host, most often by trauma (such as a prick from a rose thorn). Most patients form papules, which can progress to bullae or ulcerations. Cutaneous sporotrichosis also may be associated with ascending lymphangitis. Rare cases can disseminate, usually in immunocompromised patients.

A 73-year-old man presents to the office, complaining of "weird blisters" on his right hand, which appeared two weeks ago. He says that the rash looked like small bumps at first, then blisters appeared. He denies any trauma or known contact with sick people. He is worried because he hasn't been able to garden since the rash appeared, and he was planning on entering his roses into an annual competition this month. His vital signs are stable. On physical exam, multiple bullae with red, papular lesions are identified on his right hand and forearm, along with swollen, tender right axillary lymph nodes. What is the treatment for the cause of this man's condition? A. Potassium iodide B. Azithromycin C. Doxycycline D. Itraconazole E. Topical corticosteroids

D. Hemidesmosomes This patient has bullous pemphigoid, an autoimmune disorder characterized by IgG antibodies against epidermal basement membrane hemidesmosomes. Bullous pemphigoid can be acute or chronic and involves blister formation between the epidermis and dermis. It is considered a type 2 hypersensitivity reaction because of the formation of antibodies against hemidesmosomes. Bullous pemphigoid is similar to pemphigus vulgaris, which affects desmosomes, but is not as severe. Pemphigus vulgaris tends to affect the oral mucosa, while bullous pemphigoid mostly spares the oral mucosa. Adherens, gap, and tight junctions are other cell junctions that are not involved in patients with bullous pemphigoid.

A 75-year-old man presents to a medical clinic because of large, tense, pruritic bullae on his inner thighs and lower abdomen. Skin biopsy shows an epidermal basement membrane with immunoglobulin G (IgG) antibodies and linear immunofluorescence. What cell junction do the antibodies most likely bind to? A. Adherens junctions B. Desmosomes C. Gap junctions D. Hemidesmosomes E. Tight junctions

C. Previous radiation therapy

A 77-year-old woman presents to the emergency room with complaints of fever, malaise, and night sweats. She reports a recent enlargement of her axillary lymph nodes, which she examines weekly. She had breast cancer in the right breast ten years ago that was successfully treated with radiation and chemotherapy with no known recurrence. She reports a history of extensive travel to Africa and a 30-pack-year history of smoking. On physical exam, several axillary lymph nodes are palpable bilaterally, and smaller inguinal nodes are also noticed bilaterally. Biopsy results of the nodes are positive for non-Hodgkin's lymphoma (NHL). What is her risk factor for developing NHL? A. Travel to Africa B. Axillary lymph node involvement C. Previous radiation therapy D. Female gender E. History of breast cancer

D. KOH preparation The man's rash is most likely tinea corporis (ringworm), which is caused by dermatophytes (cutaneous fungi), such as Microsporum and Trichophyton. Potassium hydroxide (KOH) preparation is a fast and easy way to test for fungal infections in the office. The procedure involves taking a skin scraping from the infected area, adding KOH solution, and examining the specimen under a microscope. Dermatophytes have branching hyphae, which confirms the diagnosis of ringworm.

An 18-year-old man presents to the office complaining of an itchy rash on his torso that appeared one week ago. He is on the college wrestling team and is concerned that he will not be able to compete if it gets infected. His past medical history is unremarkable. Vital signs are within normal limits. Physical exam reveals an erythematous, scaly plaque with central clearing on the left lateral chest wall. Which diagnostic test would be most appropriate at this time? A. Culture on Sabouraud agar B. Culture on Eaton agar C. Culture on Thayer-Martin agar D. KOH preparation E. Wood's lamp examination


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