Hematology Exam Master 2.0

A 67-year-old man presents with a 2-day history of fatigue and shortness of breath. His history is significant for stage III colorectal carcinoma; it was diagnosed 15 months ago, and was initially treated by hemicolectomy and adjuvant 5FU/leucovorin with good result. A CT scan performed 3 weeks ago showed the presence of several small lesions in the liver. Fine needle biopsy revealed adenocarcinoma consistent with his primary cancer, and he was restarted on chemotherapy 2 weeks ago. On examination, he is pale and in obvious distress. Blood pressure is 96/62 mm Hg, and heart rate is 112. He has a well-healed midline abdominal scar and scattered petechiae over the right abdomen. Stool is positive for occult blood. Significant lab values are as follows: WBC of 1.8 x 103/uL, Hgb of 7.1 g/dL, and platelet count of 26,000/µL. What is the most likely primary cause of his anemia?

Bleeding due to thrombocytopenia (secondary to chemotherapy)

A 38-year-old woman presents with numbness and a tingling sensation in both her hands and feet (gloves and stockings) and recurrent diarrhea. Her family claims she has been "acting crazy" and abnormal. Physical examination shows an atrophic tongue (glossitis) and a neurologic examination reveals a loss of sense of vibration and fine touch. Endoscopic examination shows atrophic gastric mucosa and fasting achlorhydria. Question What findings could indicate a diagnosis of pernicious anemia in this patient?

Bone marrow shows megaloblastic erythropoiesis

A 2-year-old Caucasian boy presents with a 1-month history of decreased energy and increased somnolence. His mother admits that he is a picky eater and that he "loves to eat mattresses." Review of systems reveals that he drinks milk only with cereal and has occasional constipation. His vital signs are within normal limits. You suspect this patient may have iron deficiency anemia. Question What is the most likely diagnosis? What is the eating of non-food items called? What nail finding could be seen? What lab findings would be expected?- MVC? Hbg? Retic count? Ferritin? TIBC?

-Iron deficiency anemia -Pica -Spoon nails- Koilonychia -Labs: 6Hb (g/dl), 68 MCV (fl), and decreased reticulocyte count, ferritin low, TIBC elevate

A 38-year-old woman presents with pain in the right thigh. The pain came on gradually; she describes it as a squeezing discomfort, and it does not radiate anywhere. There have been no previous episodes, and she denies any trauma. She is otherwise well. She only takes ethinyl estradiol/levonorgestrel birth control pill (BCP) orally once daily. She admits to smoking 10 cigarettes a day. On physical exam, her blood pressure is 110/70 mmHg, pulse 72/minute, respirations 16/minute, and temperature of 98°F. Her right thigh appears more swollen than the left, and there is no skin discoloration; it is tender when squeezed. Her latest lab work is within normal limits (WNL). A Doppler ultrasound examination confirms a deep venous thrombosis (DVT) in the right femoral vein. Question What is the best long-term management for this patient?

-Warfarin (Coumadin) for 6 months only -Smoking cessation -Switching to progesterone-only BCP

A 23-year-old man presents with history of progressive fatigue; it has been increasing over the past 3 months. He feels breathless on minimal exertion. His weight registers at 140 pounds; previously, he had been steady at 189 pounds. His reduced appetite has also been worrying him. On examination, he was found to have pallor, multiple petechiae, and ecchymosis, with diffusely swollen gums. Lab investigations reveal the following: Hemoglobin - 8g/dl Total white count - 75,000/cubic ml Platelet count - 25,000/cubic ml Peripheral Smear - myeloblasts >20% with peroxidase positive Auer rods He is diagnosed with AML, with t(15;17) M3 subtype. Question The main treatment modality that can be used for the patient's condition is also used for treating what other condition?

Acne (Vitamin A/retinoic acid tx)

A 56-year-old man presents to the outpatient clinic with increased fatigability, purpura, weight loss, bone pain, and pallor. CBC was done and revealed marked anemia, thrombocytopenia, and increased white blood cell count, and differential showed marked increase in plasma cells. CT head was done and showed multiple hypodense areas in the skull bone. Question A tissue biopsy was taken and stained positive with Congo red. What is the protein stained in the specimen?

Amyloid light chain (AL) Seen with Amyloidosis

A 73-year-old woman returns to your clinic after several years for her annual physical. She has a history of mild rheumatoid arthritis, but the disease has been relatively mild and she takes no medications for it. She states that she has adjusted to living alone since her husband died. However, she has lost 30 pounds over the last 6 months, which she attributes to a loss of appetite and a lack of interest in cooking solely for herself. Labs reveal a hypochromic, normocytic anemia, low serum iron, and high ferritin. Question What is the most likely cause of her anemia?

Anemia of chronic disease

A 35-year-old man with known stage 3 chronic kidney disease due to diabetes presents with fatigue. His blood sugars are acceptable; his mood is euthymic, and he is sleeping and eating well. He does not smoke, and he has no known toxic environmental exposures. Work up shows normochromic normocytic anemia, with a hemoglobin of 11 g/dl, which is decreased from 12 g/dl 6 months ago. Medications include lisinopril, furosemide, and insulin. Iron 50 ug/dl (normal 50-150 ug/dl) Ferritin 200 ng/ml Ratio soluble transferrin receptor/log ferritin Reduced Reticulocytes 0.25% Platelets Normal Total white blood cell count 5x103/mm3 B12 levels, serum Normal Question What is the most likely diagnosis?

Anemia of chronic disease- most likely secondary to CKD

A 24-year-old woman notices that she has a bruising tendency. She frequently has numerous small bruises and purple blotches on her skin. She hates having dental work because of the associated bleeding. On physical exam, you note that she has numerous petechiae. Her lab results are shown in the chart. TEST RESULTS REFERENCE RANGE Hematocrit 42 40-50 (male) 36-44 (female) Hemoglobin 14 gm/dL 13.8-17.2 gm/dL (male) 12.1-15.1 gm/dL (female) Platelets 41,000/mm3 130,000-400,000/mm3 PT . 11.5 seconds 10.8-13.0 seconds PTT 30 seconds 25-41 seconds RBC 4.8 x 106/µl 4.5-5.7 x 106/µL (male) 3.9-5.0 x 106/µL (female) Question The patient's condition is most likely to be characterized by what?

Anti-platelet IgG synonymous with ITP?

A patient in the hospital for a wound infection is referred to you for consult. After 3 days of antibiotic therapy, the patient has gone from normal RBC results to a normocytic anemia with a slight macrocytosis (see image). The reticulocyte count is decreased. More significant is the development of a pronounced pancytopenia. A review of the charts reveals that this pancytopenia was not present upon admission. The patient has been on chloramphenicol therapy. Question What is the most likely diagnosis?

Aplastic anemia -is characterized by a normocytic anemia with a slight macrocytosis and usually decreased reticulocyte count. There is a reduction in all blood formed elements leading to a pancytopenia. Hematopoietic tissue function is effectively reduced. The reduced hematopoietic tissue function is, many times, due to a reaction to chemicals or drugs; however, neoplasia, pregnancy, infectious processes, radiation, pancreatitis, and immunological reactions can also produce an aplastic anemia.

A 14-year-old female is brought to the Family Medicine Clinic presenting with heavy menses and occasional epistaxis. The mother reports that this is common among the women in their extended family but not investigated until now. Lab values in the preliminary workup include: -Set of abnormal closure times in the PFA-100 bleeding test device (but not indicative of aspirin effect) -Normal PT -Slightly prolonged aPTT -Normal platelet count -Normal fibrinogen -Hematocrit=32% von Willebrand's workup reveals: 30% antigen level, 25% Ristocetin cofactor activity level, and 40% Factor VIII clotting activity. The patient states that she wants analgesic relief from the discomfort accompanying menstruation and desires an intervention to reduce the episodic bleeding. Question: What is the best set of recommendations you can make to this patient?

Avoid aspirin; use DDAVP (desmopressin) nasal spray at first sign of any excessive bleeding

Your patient is a 75-year-old man complaining of fatigue, weak appetite, palpitations, and weakness, as well as sore tongue, frequent stomach upsets, and indigestion problems. He also has tingling in both legs, dizziness, and started forgetting things. During the last year, he fell several times. Your examination reveals low blood pressure, swollen beefy tongue, sinus tachycardia, pallor, and loss of vibratory sensation in lower extremities. Question What diagnosis are you suspicious of ? What will be your next step in diagnosis?

B12 anemia- evidence= glossitis (beefy red tongue), weakness/neuropathy "stock and glove" (how to differentiate B12 from folate deficiency) Next step in diagnosis - Complete blood count Other useful tests: -Decrease: serum B12 -Increase: LDH, homocysteine, MMA -Methylmalanic acid increase in B12 (helps differentiate from folate deficiency)

A 29-year-old woman presents with lumps in the neck, fever, weight loss, night sweats, itching, and lethargy. She is ultimately diagnosed with stage IV Hodgkin's lymphoma and treated with chemotherapy followed by radiation. Four months later she develops shortness of breath, and chest CT scan reveals pulmonary fibrosis. Question What chemotherapeutic agent is associated with this adverse reaction?

Bleomycin

DELETE A 59-year-old man is undergoing a knee replacement and presents with abnormal preoperative laboratory results. He takes acetaminophen and over-the-counter naproxen for his knee pain but is otherwise healthy and has no other symptoms. Laboratory Findings: WBCs 3000/mm3 (N 3000-11,600) Hemoglobin 8.9 g/dL (N 13.0-18.0) Hematocrit 26.9% (N 38.5-52.0) Platelets 118,000/mm3 (N 130,000-140,000) Mean corpuscular volume 89 m3 (N 80-98) Red cell distribution width 12.6% (N 11.0-14.5) Serum vitamin B12 457 pg/mL (N 220-960) Ferritin 258 g/L (N24-336) Reticulocytes 1.5% (N 0.8-2.1) Question What diagnosis is most consistent with these results?

Bone marrow suppression

A 38-year-old woman presents with a 2-week history of sore throat and fever; symptoms have been unresponsive to over-the-counter medications. On exam, the patient appears ill. Her temperature is 39 C; Her pharynx is red and injected; you notice painful aphthous ulcers in her mouth. Complete blood count reveals a WBC of 1.60 X 109/L; differential shows 0.5% neutrophils. Hemoglobin is 9.5 g/dL. Urine dipstick shows proteinuria. Blood cultures and other analyses are pending. Question: You immediately admit the patient and order aggressive volume replacement, filgrastim, and what additional treatment?

Carbapenem Dx: Hemolytic anemia?

A 60-year-old man presents with a 1-month history of malaise and loss of appetite; he has also lost 15 lb. He denies headaches, cough, fevers, chills, and night sweats. He has no history of recent travel. His past medical history is insignificant for the past 2 years. Physical examination is significant for the presence of some skin lesions on the trunk. Palpation of the abdomen indicates hepatosplenomegaly. A CBC, electrolyte panel, and liver enzyme tests are ordered. The WBC is 150,000/uL, with a predominance of lymphocytes (90%), and the appearance of monotonous-appearing lymphocytes and smudge cells. Liver enzymes are slightly elevated, and electrolytes are within normal limits. Based on the clinical presentation and preliminary laboratory findings, a bone marrow test is ordered. Question What is the most likely diagnosis? ALL CLL AML CML

Chronic Lymphocytic leukemia Evidence: Lymphocyte dominance in diff, age of patient, abscence of blasts (r/o acute disease)

A 69-year-old man presents for an examination. He does not have a personal physician, and he has not had a physical since he was enlisted in the army. His physical examination is normal. Blood pressure is normal. He notes that he has had problems with rectal itching and burning pain. He has had a cold for 3 weeks, and it does not appear to be getting any better. Other than these 2 complaints, he has been relatively healthy. Laboratory tests are ordered and the results are shown in the table. On peripheral smear- smudge cells; fragile B cells are noted. Question Based on these test results and the clinical history of the patient, what is the most likely diagnosis?

Chronic lymphocytic leukemia CLL can be observed and with f/u and repeat CBC in 3 mo. if patient is asymptomatic

A 55-year-old man presents with a 1-week history of fatigue, night sweats, and abdominal fullness. On physical examination, you note a palpable spleen. You order a CBC; the results indicate a white blood count of 105,000 cells/mcL with a left shift of the myeloid series. The red blood cell count and morphology show anemia, and he has an elevated platelet count. To help confirm your suspicions, you order genetic studies, and the results come back with the BCR/ABL gene detected. Question What is the most likely diagnosis?

Chronic myelogenous leukemia

DELETE A 55-year-old man presents with fatigue; he has been experiencing it for a number of weeks. He also complains of a dragging sensation in the left upper abdomen. Physical examination establishes the following vitals: BP 135/90 mm Hg, Pulse 78/min, Temp 37.2 C, Resp 18/min. The spleen is found to be extended 8 cm below the costal margin and the liver extends 2 cm below the costal margin. The following laboratory tests are performed on the blood: RBC count: 3,700,000/ microliter WBC count: 3,000/microliter Platelet count: 60,000/microliter Bone marrow: inaspirable Peripheral blood smear: cells with numerous cytoplasmic projections and positive staining with tartrate-resistant acid phosphatase (TRAP) Question What is the treatment of choice for the patient's condition?

Cladribine- chemotherapy agent for leukemia

A 64-year-old man presents for a medication change. He has a history of non-valvular atrial fibrillation and has been on anticoagulation therapy with warfarin for the past 3 years, but he has had difficulty maintaining his INR in the therapeutic range. After thorough counseling and assessment, he is considering another oral anticoagulant medication that has a reversal antidote in case he develops life-threatening bleeding. Question What agent is most appropriate in this patient?

Dabigatran -DOAC

Your patient is a 65-year-old man with progressive fatigue. His fatigue started -7 months ago, and it is worsening; sometimes, he feels "too tired even to get out of bed". Over the last month, he has started having bone pain, and he has stopped his usual evening walks because of a decreased exercise tolerance. He has also noticed that he is sweating at night. At the same time, he has started having a feeling of abdominal fullness and early satiety; it is accompanied by a constant sensation of bloating, even after a small sandwich or an apple. He has lost approximately 5 kg over the last 6 months. Physical examination reveals liver palpable about 4 cm below the right costal margin, and the spleen is palpable about 8 cm below the left costal margin; the rest of his physical examination is within normal limits. His laboratory analyses are below: Question: What is the patients most likely diagnosis What will be your therapeutic approach? A. Watchful waiting B. Immunomodulatory agents (Thalidomide) C. Splenectomy D. Androgen (Danazol) E. Splenic radiation F. Anti-gout agent (colchicine) G. Tyrosine kinase inhibitor (Ruxolitinib)

Dx: CML Clinical pearls for CML: increase leukocytosis, splenomegaly= most common physical exam finding, many patients may be asymptomatic, LAP (leukocyte alkaline phosphatase) score decreased, cytogenic analysis/ FISH= Philadelphia chromosome- translocation between 9:22 G. Tyrosine kinase inhibitor (Ruxolitnib) Other medications commonly used include- Gleevec (Imatinib, Dasatinib, Nilotinib --> associate with "nib" drugs

A 72-year-old man, who is known to have adenocarcinoma of lung, s/p left upper lobe wedge resection and chemoradiation presents with left flank pain, dysuria, and difficulty voiding. Urine examination shows moderate RBCs. Renal ultrasound is unremarkable. The patient has a history of chronic DVT and is currently on enoxaparin (low molecular weight heparin). You have been consulted for a low platelet count (29,000/mcL). His other pertinent laboratory tests include LDH- 570; heparin-induced antibody tested negative, PT/aPTT 15.7 and 35.1 in addition to those listed below. Hb count 8.1 gm/dl PT 15.1 (10.5-14.0) PT mix 12.8 (corrected) aPTT 54.7 (22-34.0) aPTT mix 29.9 (corrected) Thrombin time 20.2 (16.0-23.0) Fibrin degradation products 320 (0.0-2.5) Fibrinogen 102 (150-350) D-dimer 64.0 (0.0-0.4) AT-III 103% (80-120%) Schistocytes- Occasional Platelet count- 29,000/mcL (decreased) Bilirubin 1.2 mg/dl Cr 1.5 mg/dl Question What is the diagnosis? What laboratory findings support your suspicions?

Dx: Disseminated intravascular coagulation Labs consistent with DIC include" -Elevated FDP and DD-dimer -Prolonged PT/aPTT -Reduced fibrinogen -Thrombocytopenia.

A 28-year-old woman receiving her first blood transfusion begins to experience flushing, tightness in her chest, and difficulty breathing within minutes of the start of the infusion. Her blood pressure drops rapidly, and the transfusion is immediately discontinued. Epinephrine is administered with good effect. Specimens sent to the lab show evidence of intravascular hemolysis. Question What is the name of the rxn? What is the most likely cause of such a reaction?

Dx: Hemolytic Transfusion Reaction Cause- An ABO incompatibility -Can occur within minutes think ABO and intravascular hemolysis STOP TRANSFUSION IMMEDIATELY, report to blood bank

A 17-year-old boy is being investigated through the outpatient department due to a 6-month history of repeated episodes of fever, night sweats, and lethargy. He has also lost a substantial amount weight. The examining physician notices slight pallor and finds that the cervical lymph nodes are palpable, discrete, and non-tender. Routine blood test shows lymphocytic preponderance and increased erythrocyte sedimentation rate. The histology also reveals some cells with mirror-image nuclei. Chest X-ray is normal. The diagnosis is confirmed through node biopsy. Question: What is the likely diagnosis? What statement concerning this disease is false? -It may be related to a viral infection -It has a bimodal age appearance -Reed Sternberg cells on can be seen on peripheral smear -The most common early initial sign is painful cervical/ supraclavicular lymphadenopathy

Dx: Hodgkin's Lymphoma -The most common early initial sign is painful cervical/ supraclavicular lymphadenopathy -It is marked by PAINLESS lymphadenopathy -The viral infection most commonly associated with Hodgkins is EBV

A 45-year-old man is evaluated for a 6-month history of palpitations, easy fatigability, and chest pain on exertion. He does not smoke or consume alcohol; he has no significant past medical history. His body weight has remained stable. He has spent the last 2 years traveling the world. His wife says that he eats "unhealthily." He is a strict vegan, consumes a lot of snacks, and has never taken any vitamin supplements. Examination shows a 5'7" male with a BMI of 19. His BP is 130/70 and pulse 90/min; his temperature is 98.4°F. Conjunctival pallor is present. Auscultation shows a grade 2/6 murmur ejection systolic murmur heard all over the precordium. Lab investigations are sent and are given below: Hb: 8.8 g/dL WBC: 6000/mm3 MCV: 72 Ferritin: low TIBC: elevated Stool occult blood: negative ESR: 8 mm/hour TSH: normal Question What is the most likely dx? What is the most appropriate medical therapy?

Dx: Iron deficiency anemia- evident by low Hbg, microcytic (low MVC <80), low ferritin and high TIBC Tx: Oral ferrous sulfate

A 60-year-old man presents to the office for routine follow-up of hypertension. A CBC reveals: WBC 12.0 x 103/mm3 4.5 - 11 RBC 7.0 x 106/μl 4.6 - 6.2 (male) Hematocrit 60 % 40 - 54 (male) MCV 68 μ3 80 - 100 RDW 14.9 % 11.6 - 14.6 Platelets 550,000 mm3 150,000 - 450,000 On examination, blood pressure is 160/88 mm Hg, pulse is 86/min, temperature 97.6°F, and respirations 18/minute. His skin appears ruddy and the tip of the spleen is palpable. Pulse oximetry reveals a saturation of 99%. Question What is the most likely diagnosis What distinguishing finding would you expect on history or physical exam? -Recurrent epistaxis -Hemarthrosis -Pruritis after a hot shower -Tinnitus

Dx: Polycythemia Vera- elevation of all three myeloid lines (RBC, WBC, PLT) Pruritis after a hot shower= common finding

A 52-year-old Caucasian man presents with easy fatigability, poor concentration, and "stronger than usual" effort-related palpitations. He denies any constipation or weight loss. His past medical history includes type II diabetes, which was diagnosed 10 years ago; it is well controlled with diet and metformin, and there are no complications. Both his parents are in good health; his sister had a malignant breast nodule removed 2 years ago, but she showed no signs of metastatic disease. His physical examination shows mild obesity (BMI 32 kg/m2), but it is otherwise normal. A laboratory workup shows: -Glucose 91 mg/dL -Na+ 138 mEq/L -K+ 4.9 mEq/L -Cl- 100 mEq/L -Hemoglobin 11 g/dL -Hematocrit 32% -Mean corpuscular volume(MCV) 82 fl -Mean corpuscular hemoglobin concentration (MCHC) 24 pg/mL -Leukocytes 7,400 /mm3 w/normal differential -Platelets 310,000 /mm3 (150-450) -Ferritin 29 ng/L (25-300 ng/mL) -Total iron binding capacity of 450 ng/dL(250-450) Question What is the most appropriate next step in diagnosis?

Fecal occult blood testing (FOBT) Patient shows evidence of mild iron deficiency anemia NEW ONSET IDA IN OLD PT IS COLORECTAL CANCER UNTIL PROVEN OTHERWISE

A 16-year-old boy presents for a wellness examination; he is doing well academically and socially. Past medical history is significant for hereditary spherocytosis, diagnosed at age 3. His anemia has usually been moderate, with his lowest hemoglobin at 12.5 gm/dL. He has required a transfusion on one occasion at age 7 due to a hemolytic crisis brought about by an acute viral infection. Growth and development have been normal, and immunizations are current. Vital signs are normal. The spleen is palpable 2 cm below the left costal margin and there is mild icterus. Question What chronic treatment should this patient receive? -Vitamin D -Vitamin B12 -Folate -Iron

Folate Required to sustain erythropoiesis

An 11-year-old boy presents with a chronic history of mild hemolytic anemia, intermittent jaundice, and right upper quadrant pain. He denies any shortness of breath, muscle aches, or joint pain. Question Supplementation with what should be given to the patient as part of the treatment plan? Vitamin B12 Folic acid Vitamin A Cobaulmin

Folic acid

DELETE A 64-year-old man of Jewish descent presents at the ER with a history of progressive fatigue over several years and increasing bone pain. He has firm splenomegaly and pancytopenia. Bone marrow aspiration done later on during the investigation shows infiltration with large cells with fibrillar cytoplasm. Question What is the most likely diagnosis?

Gaucher's disease

An 18-year-old woman presents with excessive fatigue that has been occurring for the past 4 months. She denies any specific health concerns or recent illnesses. She is taking an oral contraceptive and reports that her menstrual bleeding is light in flow. Recent laboratory findings include a TSH level of 1.44 U/mL (N 0.5-5.0), a hematocrit level of 38% (N 36-46), a mean corpuscular volume of 71 (N 78-102), an RBC count of 5.7 million/mm3 (N 4.10-5.10), and ovalocytes on a peripheral smear. Question Which one of the following tests is mostly like to help explain this patient's initial laboratory abnormalities and lead to a diagnosis?

Genetic testing

A 45-year-old man is referred to your hematology office by his primary care physician. He had a physical examination 2 months ago; routine blood tests revealed marked elevation of serum iron levels. Follow up testing revealed elevated transferrin saturation of greater than 60%, as well as elevated ferritin. The iron studies were repeated twice for confirmation; both were fasting specimens. The patient reports that he has been feeling well, and has not had any unusual or unexplained symptoms. He reports that he eats a well-rounded diet. He is not a vegetarian, but he does not feel that he eats excessive amounts of red meat. He does not take any vitamins or iron supplements. He had not had a physical in years, but does not recall anyone previously mentioning elevated iron levels to him before. He has no history of hemolytic anemias, multiple transfusions, liver disease, or hemodialysis. He has never been hospitalized. He has never had any surgery. He is not currently taking any prescription medications. He is allergic to sulfur, which causes a rash. Family history is significant for kidney failure and heart failure in his grandfather, but he is not sure what caused this. He is not aware of anyone in his family with hypertension, diabetes, cancer, or stroke. Social history reveals that he is a banker, and he works in an office setting. He is not exposed to any toxic chemicals, fumes, or smoke. He does not drink alcohol. Review of systems is negative for chest pain, shortness of breath, abdominal pain, rectal bleeding, change in appetite, or weight loss. Physical examination reveals a man that appears to be his stated age. He is well developed. Blood pressure is 110/70 mm Hg, pulse 74/min, and respirations 12/min. He is afebrile. Head, ears, eyes, nose and throat examinations are normal. Lungs are clear to auscultation bilaterally. Cardiac auscultation reveals S1, S2, and possibly an S3. There is no distinct murmur, and the rhythm is regular. Abdominal examination reveals no tenderness. There are normal bowel sounds. The liver span is slightly increased to percussion. There are no abdominal bruits. There is no peripheral edema. Peripheral pulses are normal, and neurologic examination is nonfocal. Laboratory studies reveal: White blood cell count 8.5 Hemoglobin 14.7 Hematocrit 50.2 Platelets 177 Serum iron 202 (high) Serum ferritin 703 (high) Transferrin saturation 62% Question You discuss hemochromatosis with the patient, but recommend a liver biopsy to confirm the diagnosis and help determine the prognosis. The patient refuses to accept the risk of having a liver biopsy to confirm the diagnosis; based on his own research, he is convinced that he has hemochromatosis. Before discussing treatment options, how could you also confirm the diagnosis of hemochromatosis?

Gradual phlebotomy of 16 units of blood, then recheck indices of erythropoiesis

A 24-year-old woman is evaluated in the endocrinology clinic. She is on 100 mcg of thyroxine per day for treatment of Hashimoto's thyroiditis and receives oral steroids for autoimmune adrenal insufficiency. In addition, she complains of fatigue, numbness, and tingling in her feet. When routine labs were drawn, the following values were noted: Hb: 9.9 g/dL MCV: 102 Iron indices: normal Folate: normal B12: low Parietal cell antibodies: high Question What is the likely diagnosis? What would prevent the development of megaloblastic anemia in this patient?

Hashimoto's Disease is autoimmune, therefore chance of another autoimmune condition is likely. Patient may be at risk for development of pernicious anemia- which can cause a macrocytic anemia, B12 deficiency. Preventative treatment include: Intramuscular injection of B12

A 50-year-old man presents with weakness and abdominal discomfort. Upon questioning, he acknowledges a lack of sexual desire. He denies any photosensitivity. On physical examination, his liver is enlarged. He has an abnormal skin pigmentation that is located on his face, neck, and elbows; it gives his skin a slate-gray hue. His laboratory results are in the chart. TEST RESULTS REFERENCE RANGE TIBC 275 μg/dL 250 - 375 μg/dL Plasma iron 220 μg/dL 50 - 150 μg/dL Transferrin saturation 90% 20 - 40% Question What is the most likely diagnosis?

Hemochromatosis

A 34-year-old woman presents with fatigue, weakness, shortness of breath, nosebleeds, bruising, and abdominal pain. On physical exam, her skin is pale; you note abdominal tenderness, an enlarged spleen, sternal tenderness, and an irregular, rapid heartbeat. Labs reveal an anemia, high reticulocyte count, and an elevated bilirubin level. Peripheral blood smear reveals schistocytes. Question What is the most likely diagnosis?

Hemolytic anemia

A 12-year-old Jewish girl is brought in by her father. The father reports that the child started to bleed profusely following tooth extraction 2 hours ago. Further questioning reveals that there is a history of bleeding disorder in the child's maternal uncle and aunt. Examination reveals tachycardia and bleeding from the site of tooth extraction. Coagulation profile reveals prolonged activated partial thromboplastin time (aPTT), normal prothrombin time (PT) and thrombin time (TT), and deficiency of factor XI. The child was treated with fresh frozen plasma (FFP) and fibrin glue. Question What is the likely diagnosis? Hemophilia A Hemophilia B Hemophilia C von Willebrand Disease

Hemophilia C- most common in Ashkenazi Jews, deficiency in factor XI Hemophilia A- deficiency in factor XIII Hemophilia B- deficiency in factor IV vWD- deficiency in vWF and factor XIII

A 22-year-old girl presents with sudden onset of diffuse abdominal pain; it is accompanied with malaise, vomiting, thirst, irritability and the appearance of dark-colored urine. The symptoms appeared 2 days ago when she was hiking in the mountains with her friends. During that period of time, she was exposed to rather strenuous physical effort; food intake was irregular and inadequate. She experienced similar symptoms 5 years ago; a diagnosis of acute intermittent porphyria was established, but the attacks of the disease did not recur again until now. Physical examination reveals a restless, dehydrated, averagely nourished person; there is also sinus tachycardia (heart rate 97 beats/minute) and epigastric tenderness on palpation. Blood pressure is 140/95 mm Hg, and Chvostek's sign is positive; the remainder of the physical examination is normal. During the examination, she suddenly develops a seizure. Question What electrolyte abnormality is most likely to result in seizure in the patient with acute intermittent porphyia? Hyperkalemia Hyponatremia Hypercalcemia Hyperphosphatemia

Hyponatremia

A 62-year-old man presents with a 6-month history of a painless mass on the left side of the neck. He denies any preceding or concurrent illness and is currently afebrile. In fact, recent history is significant only for weight loss from 180 to 160 pounds over the last several months. Past history is significant for severe seasonal allergies. On physical examination, he has left-sided cervical and supraclavicular lymphadenopathy consisting of matted nodes that is painless to palpation. Excisional biopsy of a group of nodes reveals a malignant lymphoma, which is classified as follicular, small-cleaved cell. Chest X-ray demonstrates left-sided hilar adenopathy. A CT scan of the abdomen and pelvis is negative; so is a bone marrow biopsy. Question According to the Ann Arbor system, what stage would this lymphoma be classified as?

IIB

A 67-year-old man had a lung transplantation for end-stage COPD 2 years ago. He now presents with a sudden onset of altered mental status and fever. The patient's current medications include FK-506, cyclosporine, azathioprine (Imuran), and prednisone. Stat blood tests in the ED are shown in the table. A CT scan of the brain without contrast is unremarkable. CXR shows no pulmonary parenchymal infiltration. A quick examination of peripheral blood smear shows many schistocytes. A preliminary diagnosis of thrombotic thrombocytopenic purpura (TTP) is made, and plasmapheresis is started. Question What is the most likely coagulation profile of this patient? Will PTT or PT more likely be prolonged ?

INR 1.0, APTT 29 sec

A 67-year-old man is recovering following an uncomplicated right total hip arthroplasty. His past medical history is significant for hypertension and mild asthma. On postoperative day 2, he begins to note stiffness in his right leg. On exam, his right calf is noted to be swollen and slightly warmer than the left leg. The remainder of the exam is unremarkable. He is afebrile and his vital signs are stable. A likely diagnosis is made. The patient is given a bolus followed by continuous infusion of unfractionated intravenous heparin. He is discharged on oral warfarin therapy. Question What should be the target international normalized ratio (INR), and for how long should therapy be continued?

INR 2.0 - 3.0 continued for 3 - 6 months

A 38-year-old woman presents with increased menstrual bleeding over the course of the past several months; she has also had 2 episodes of epistaxis within the last week. Other than mild fatigue, she relates no other symptoms. She is afebrile and normotensive. Physical exam is significant only for scattered petechiae on the lower extremities. Except for an Hgb of 11.4 g/dL and a platelet count of 40,000/uL, complete blood count is within normal limits. Peripheral blood smear is normal. Question What is the most likely diagnosis? TTP ITP DIC AIHA

Idiopathic (Immune) thrombocytopenic purpura

A 63-year-old woman presents with weakness, shortness of breath, and palpitations. She also notes a dragging sensation in the left upper abdomen. She had a modified radical mastectomy for carcinoma of her right breast. Physical examination reveals moderate pallor, mild pedal edema, and hepatosplenomegaly. She is afebrile, and her pulse and blood pressure are within normal limits. There is no history of chest pain. The CBC and peripheral blood smear give the following information: Hemoglobin 9 gm% Red cell morphology: normocytic normochromic anemia with anisopoikilocytosis, teardrop-shaped cells, nucleated red cells, and polychromatophils. Total WBC count: 21,000/mm3 Differential count revealed a shift to the left with presence of myelocytes and promyelocytes in small proportion. Platelet: 500,000/mm3 with the presence of giant platelets and platelets with abnormal granulation. Based on these results, you advise a bone marrow examination. The marrow biopsy has the following findings: Cellular marrow with granulocytic and megakaryocytic hyperplasia Granulocytes show hyperlobation, nuclear blebs, and nuclear-cytoplasmic asynchrony Giant megakaryocytes and micromegakaryocytes Silver stain shows a marked increase in reticulin fibers Question What is the most likely diagnosis?

Idiopathic myelofibrosis

A 45-year-old man is evaluated for bleeding gums. He notices easy bleeding with minor trauma, especially after brushing his teeth. He also bruises easily and has multiple ecchymoses. He is a non-smoker and drinks no alcohol. Physical examination shows BP 110/70; BMI 18; Pulse 70/min; Temp normal. Cardiovascular and respiratory exam appear normal. Abdominal exam shows an enlarged liver and spleen, with the spleen measuring more than 10 cm in diameter. Lab investigations are shown below: Hb: 7.2 WBC count: 76,000 Neutrophils: 55% Lymphocytes: 20% Eosinophils: 12% Basophils: 10% Monocytes: 3% Platelet count: 42,000 LAP: low Peripheral smear: numerous granulocytes, with immature myeloid cells (myelocytes and metamyelocytes), and occasional blasts. Bone marrow appears hypercellular, with increase of the myeloid cell line (neutrophils, eosinophils, basophils) and progenitor cells. Megakaryocytes are prominent and increased. Cytogenetic studies show reciprocal translocation between chromosomes 9 and 22. Question What is considered to be the treatment of choice? Steroids Observation Imatinib Methotrexate

Imatinib- is a tyrosine kinase inhibitor used for the treatment of CML

An 18-year-old man presents to a university health clinic with a very sore throat and headache. He has had severe pharyngitis for about 12 days. He has felt exhausted and anorexic for 3 to 4 weeks. On physical exam, he has a temperature of 40 degrees C and his BP is 118/75 mm Hg. On palpation, his spleen is slightly tender and moderately enlarged. Blood work shows relative and absolute lymphocytosis; it also shows some atypical cells. Most of the lymphocytes are identified as T cells. Question Based on these findings, what is the diagnosis?

Infectious mononucleosis

A 45-year-old woman has a past history that includes the fracture of both femur bones in a car accident 6 months ago. She presents in the emergency department with frank hematuria that started suddenly the day prior to presentation. It is painless, and it is not associated with loin or abdominal pain. The patient reports taking warfarin that was prescribed for her 1 month ago; she had developed deep vein thrombosis (DVT). On examination, the patient is pale, anxious, and has cold extremities. Her blood pressure is 100/70 mm Hg, and her pulse is 100 beat/minute. Her cardiac examination reveals accentuated heart sounds, no added sounds, and no murmurs. Question What is the most important lab investigation at this time?

International normalized ratio (INR) The international normalized ratio (INR) is a calculation based on results of a PT and is used to monitor individuals who are being treated with the blood-thinning medication (anticoagulant) warfarin (Coumadin®). The PT and INR are used to monitor the effectiveness of the anticoagulant warfarin

A 34-year-old woman presents with fatigue, headache, shortness of breath, and dizziness upon rising from a chair. The patient denies pregnancy or recent illness; she is taking no medications. She has no pain. Upon physical exam, her skin is pale; her nails are brittle, and she has a blood pressure of 98/76 mm Hg. CBC reveals a microcytic anemia. Question What is the most likely diagnosis?

Iron deficiency anemia

A 42-year-old African-American woman presents with increased fatigue and unusual cravings for chalk and raw potatoes over the past few months. Question What is the most likely diagnosis?

Iron deficiency anemia

A 50-year-old man presents with weakness and abdominal discomfort. Upon questioning, he acknowledges a lack of sexual desire. He denies any photosensitivity. On physical examination, his liver is enlarged. He has an abnormal skin pigmentation that is located on his face, neck, and elbows; it gives his skin a slate-gray hue. His laboratory results are in the chart. TEST RESULTS REFERENCE RANGE TIBC 275 μg/dL 250 - 375 μg/dL Plasma iron 220 μg/dL 50 - 150 μg/dL Transferrin saturation 90% 20 - 40% Question: What is a common complication of this condition?

Iron deposits in liver leading to --> Hepatocellular carcinoma

A 16-year-old male high school football player was tackled and landed on the football on his left side. He is brought to the emergency department and is found to have nausea, diaphoresis, tachycardia, hypotension, left upper quadrant tenderness, and pain in the tip of the shoulder while lying down with his legs elevated. A CT is done, and he is found to have a ruptured spleen. Question What is the name of the diagnostic finding most often associated with this patient's illness? Kehrs sign McBurneys test Cullens sign Lachmans test

Kehr's sign

A 2-year-old boy presents with his mother due to her concerns about his paleness; she states that he used to be playful, but now looks tired and doesn't even cry as he did previously. She first noticed this occuring 3 months after moving to his grandmother's old house. CBC is ordered and shows the following results: RBC 4 million/microliter WBC 11,000/mm3 Hb 9 mg/dL Platelets 180,000/mm3 Hematocrit 31%. Blood smear shows basophilic stippling Question What is the most likely diagnosis?

Lead poisoning

A 77-year-old Greek woman has been experiencing anorexia and weakness for 3 months; she presents after a syncopal episode. She appears pale on physical exam, but she is alert. Her pulse is 110/minute, respirations 22/minute, blood pressure 170/70 mm Hg (supine), 120/60 mmHg (standing). Her lab work is inclusive of hemoglobin = 4.3 g/dL; hematocrit = 14%; white blood cells count = 7.5x109/L; platelets = 452 x 109/L; mean corpuscular volume = 60fl; mean corpuscular hemoglobin concentration = 29 g/dL; red cell count of 1.6 million/uL. The blood smear demonstrates hypochromic, microcytic red blood cells, a few target cells, and profound red blood cell heterogeneity. Question What is the likely diagnosis? What is the next step in the evaluation of the patient?

Likely dx= Beta Thalassemia minor- Beta seen MC in Mediterranean descent Next step-Serum ferritin level (r/o Iron deficiency anemia) -Rule in thalassemia with Hgb electrophoresis- (+) HbH

A 50-year-old man presents with vertigo while shopping in the mall yesterday. The episode lasted for a few seconds and was not accompanied by tinnitus, fall, loss of consciousness, or visual/auditory disturbance. He has no history of similar symptoms in the past. He was diagnosed with a peptic ulcer 4 months ago and is being treated for the same. He has been a smoker for the past 15 years and is allergic to pollen but does not have any other known allergies. He has no other significant medical or surgical history. His mother was diagnosed with asthma and his father died of a blood cancer, the details of which are not known. He has lived in the region of the Rocky Mountains for 20 years, is married, and works in dairy farming. On examination: weight is 180 lbs, and height is 5 feet 8 inches; pulse is 80/min, BP is 150/90 mmHg, and temperature is 98.8 °F. His eye and ear examination is normal. The rest of the systemic examination, including neurologic exam, is normal. His oxygen saturation is low, and serum erythropoetin levels are elevated. Other test results are as follows: hemoglobin is 18g/dl; carboxyhemoglobin is normal; hematocrit is >55%; white blood cells are 4.2 thou/cu mm; platelets are 232 thou/cu mm; lymphocytes % is 43.1; monocytes % is 7.8; neutrophils % is 43.7; eosinophils % is 4.8; and basophils % is 0.6. Question What is the most likely underlying cause of this patient's condition?

Living at a high altitude

Sorry, I am not a pathologist A 60-year-old male presents to your office with fatigue and generalized lymphadenopathy. Physical examination reveals massive splenomegaly in addition to lymphadenopathy. A lymph node biopsy was subsequently performed. The image represents a histologic section of the lymph node (refer to the image). The most likely diagnosis is:

Mantle cell lymphoma - a type of non-Hodgkin's Lymphoma Lymphadenopathy in Non-Hodgkins= more generalized compared to Hodgkins where lymphadenopathy presents more commonly in upper body

DIDNT LEARN A 2-year-old severely ill girl with a 2-day history of irritability and pyrexia presents with her mother. Today the girl started vomiting, is drowsy, and has deteriorated rapidly during the day. In addition, her mother noticed a rash today which has been spreading extremely quickly over the girl's body. Physical exam reveals a pyrexial, very drowsy girl covered with a petechial rash; the rash is more prominent and largely purpuric and hemorrhagic on the extremities. Refer to the image. What is the most likely initial diagnosis for this patient based on the rash and the clinical picture?

Meningococcemia

An 84-year-old widow presents with a history of osteoarthritis, gastroesophageal reflux, anemia, and very mild dementia. The patient lives alone; her daughter, who has brought her in, visits every 1 - 2 weeks to help with shopping and housework. Until now, the patient has been able to manage fairly well; lately the daughter feels her mother is becoming increasingly confused. Although there is adequate food in the house, she suspects her mother is not eating properly and may not be taking her medications. The patient states she is 'fine' except that her feet "always feel tingly, like they are asleep," She states that she eats enough for herself, but is vague when asked about her medications; they include a multivitamin, pantoprazole (Protonix) and donepezil (Aricept) daily, and alendronate (Fosamax) weekly. Her daughter notes that her mother is not known to drink alcohol. except for an occasional glass of wine with dinner. The patient's physical exam is generally unremarkable; the exception is her neurologic exam which is significant for mild ataxia and decreased vibratory and proprioception in her hands and feet. There is no nystagmus. Mini-mental state examination score is 23. Her laboratory studies are as follows: WBC 8.6 x 1,000 cells/cmm; RBC 3.41 x 100,000 cells/mcL; HCT 35.1 L%; MCV 100.6 fL; MCHC 34.4%; PLT 179,000/mcL; B12 208 pg/m (211 - 911 pg/mL); and folate 4 ng/m (5.4 - 24 ng/m). Question What single lab result, if elevated, would best correlate with the patient's symptoms?

Methylmalonic acid (MMA) MMA is elevated in B12 deficiency and normal in folate deficiency Another way to differentiate between these two is by the presence of neuropathy/neurologic sx in B12

A 29-year-old man presented 3 days ago with a sore throat and fever; he was treated with amoxicillin and now presents with generalized fatigue, abdominal pain, and a maculopapular rash. The patient is sexually active with one partner who was sick 1 month ago; she is now better. Question What is the most important single test to be done now?

Monospot heterophile antibody test

A 62-year-old woman presents with severe pain of her ribs. She claims to have developed the pain immediately after having sneezed forcefully the previous night. She also describes a generalized joint pain throughout her body. She denies any cough and fever. Her past medical history is not significant for any recent travel or illness. Radiographs of her ribs are significant for fractures and what appeared as "punched-out lytic lesions" on the sternum. A CBC is significant for a normocytic-normochromic anemia, rouleaux formation of red blood cells, and the occasional presence of the following cell (refer to the image) on the differential. Serum globulin is elevated. The patient is positive for Bence Jones proteinuria. Question What is the most likely diagnosis? Non-Hodgkins Lymphoma Hodgkins Lymphoma Multiple Myeloma CML

Multiple myeloma Evidence- bone pain, "punched-out" lytic lesions on imaging, normocytic normochromic anemia, rouleaux formation, Bence Jones proteins urine electrophoresis

A 62-year-old man was diagnosed with multiple myeloma 1 year ago; he is currently hospitalized with intractable nausea and vomiting. Since he was asymptomatic at the time of diagnosis, he has not been treated for his cancer. His wife reports he has not eaten in several days, but he is constantly drinking water. He appears confused and lethargic. When adjusted for the albumin level, serum chemistries reveal an elevated level of calcium (12.0 mg/dL). Question What should the initial treatment of his hypercalcemia include?

Normal saline

A 5-year-old boy presents because of repeated bruising with ecchymoses and prolonged bleeding. 2 days ago, a minor cut in his knee bled for 2 hours and only improved after application of a compressive dressing. He has received all immunizations, reached all developmental milestones at appropriate ages, and has no relevant past medical history. His mother and 8-year-old brother are both in good health. Vital signs are within normal range. On examination, he has diffuse ecchymoses on his hands, knees, and legs. There is no palpable lymph node enlargement. His liver is palpable 1 cm below the right costal border and has a smooth texture and outline. There are no signs of splenomegaly. A laboratory workup shows the following: Ht 39% Hb 13 mg/dL, MCV 89 fl, platelets 31,000/mm3. Liver function tests were as follows: AST 14 U/L, ALT 12 U/L, γGT 22 U/L. Total bilirubin 0.5 mg/dL, direct bilirubin 0.2 mg/dL. A peripheral blood smear shows normal erythrocytes and leukocytes, a reduction in the number of platelets, and a few giant platelets. Question What is the most appropriate next step?

Observation only.

A 35-year-old man with stage 3 chronic kidney disease has had a decreasing hemoglobin count from 12 g/dl to 10.5 g/dl over the past 18 months. He is asymptomatic. Work up shows: Hemoglobin 10.5 g/dl Iron 49 ug/dl (normal 50-150 ug/dl) Transferrin Saturation 18% Ferritin 75 ng/ml Stool occult blood Negative, 3 samples B12 Normal Folate Normal TSH Normal Reticulocytes Low Question What treatment is suggested?

Oral iron supplements

Not learned in class, included in PANCE pearls You examine a 32-year-old man who was admitted 2 days ago for deep vein thrombosis (DVT). This is the second episode of DVT at the right calf; the first one being recorded 8 months ago. Neither episode revealed any risk factors or familial associations. On examination, the right calf is edematous, tender, and painful on palpation. There is 3 cm increase in the circumference of the right calf compared to the left calf and the maneuver of dorsiflexion of the right ankle with the knee extended produced pain in the calf. The patient has jaundice and tachycardia. Blood analysis reveals anemia with high reticulocyte count, elevated levels of LDH and indirect bilirubin, low haptoglobin level, and a positive result from Ham test. Duplex vascular echography reveals a thrombus at the right popliteal vein. Question What is the most likely etiology of this patient's condition?

Paroxysmal nocturnal hemoglobinuria Ham test= blood test to dx PNH

Did not learn in class- is in PANCE pearls A 42-year-old man presents to your office for his annual exam; he is found to be anemic with thrombocytopenia. He does not smoke or drink, and he is sexually active. He has 2 children in their teens. He is experiencing erectile dysfunction on occasion, and he also occasionally experiences severe abdominal pain. He has had several episodes of esophageal spasm in the past year, and on 1 occasion had to go to a local ED due to the inability to swallow. He was treated and released and told to follow up with his family doctor because he had blood in his urine. He states he has noted blood in his urine upon rising in the morning 3 or 4 times over the past 3 months. He has no history of acute illness after exposure to mothballs, sulfa-based drugs, or fava beans. Physical exam reveals a healthy-looking man with normal vital signs and mild scleral icterus. There is no organomegaly and no significant lymphadenopathy. His LDH is elevated and his CBC shows a normocytic anemia with low platelets and normal WBC. He has an unconjugated hyperbilirubinemia and a low haptoglobin level. His reticulocyte count is elevated. Question What is the most likely diagnosis?

Paroxysmal nocturnal hemoglobinuria Rare acquired, life-threatening disease of the blood. The disease is characterized by destruction of red blood cells (hemolytic anemia), blood clots (thrombosis), and impaired bone marrow function (not making enough of the three blood components) The classic sign of PNH is red discoloration of the urine due to the presence of hemoglobin and hemosiderin from the breakdown of red blood cells. As the urine is more concentrated in the morning, this is when the color is most pronounced.

A 65-year-old woman presents with left-sided chest pain. She experienced a sudden onset of sharp and stabbing pain over the left side of her lower chest. The pain is aggravated on deep breathing and movements. There is no radiation of the pain. Her past medical history is significant for hypertension, for which she takes a diuretic daily. She was hospitalized 5 months ago for replacement of her left knee joint. She smokes 1 pack of cigarettes daily and drinks a glass of wine on occasion. Her temperature is 37.2° C, blood pressure is 120/62 mm Hg, heart rate is 108/min, and respiratory rate is 25/min. Her pulse oximetry reveals oxygen saturation of 92% on room air. On auscultation of the chest, there are coarse rales over the left lower chest. Her heart exam reveals normal heart sounds with a loud pulmonary component of the second heart sound. The chest X-ray shows atelectasis of the left lower lung. CT pulmonary angiogram reveals a pulmonary embolism. The patient is started on intravenous unfractionated heparin. APTT is monitored daily and is within therapeutic range. She is also started on warfarin on day 3; on day 5, she reports pain, pallor, and abnormal sensations in her left arm. On examination, her left arm is pale and tender with diminished pulses. Lab results: Hb: 12g/dL Hematocrit: 42% Leukocytes: 10,000 Platelets: 60,000 INR: 1.1 APTT: 60 sec PT: 16 sec Question What is occurring in this patient What is the next step in managing this patient?

Patient is experiencing heparin-induced thrombocytopenia (HIT) Discontinue unfractionated heparin and warfarin ASAP Start a direct thrombin inhibitor (hirudin [lepirudin], bivalirudin, and argatroban)

A 4-year-old boy begins to develop hematomas. He mentions knee pain to his mother, and she notices his knees are swollen and painful. His mother is very concerned because she knows her father had a bleeding disorder. Question What is the likely diagnosis? What laboratory test is most likely to be abnormal in this patient?

Patient most likely has hemophilia Hemophilia A= factor 8 deficiency Hemophilia B= factor 9 deficiency Intrinsic pathway- factors 8,9,11,12 Extrinsic pathway- factor 7 Instrinic pathway lab measurement= PTT Extrinsic pathway lab measurement PT Therefore, the lab test to order for suspected hemophilia would be a PTT- test would be prolonged

A 46-year-old man with a history of IV-drug abuse and AIDS presents with recurrent acute rectal bleeding. Physical examination shows a cachectic man with bilateral swelling of lower extremities with multiple superficial skin ulcers. He has a temperature of 38.5°C and non-specific ST-T wave changes over lateral leads. Digital rectal examination showed a bleeding hemorrhoid. He remains asymptomatic from bleeding without orthostatic changes, but he is found to have some minor changes in mental status, including loss of coordination. Initial laboratory tests are shown in the table. Peripheral smear showed a moderate number of schistocytes, in addition to nucleated red blood cells, neutrophil toxic granulation, polychromasia, etc. He is later admitted to rule out myocardial infarction, care for his rectal bleeding, and receive transfusion support. Because of his elevated temperature, blood and urine cultures are obtained. The patient is started on empiric antibiotics with vancomycin and ampicillin/sulbactam. Question Based on the likely diagnosis, what is the most appropriate next step in the management of this patient?

Plasmapheresis (evidence of hemolysis: elevated indirect bilirubinemia, high LDH, nucleated red cells in peripheral smear, in addition to schistocytes, marked anemia; impaired renal function; fever, changes in mental status, thrombocytopenia, and normal coagulation profiles)

A 68-year-old woman presents with a 2- to 3-month history of dyspnea on exertion, easy fatigability, and lassitude. She states that recently she has been having difficulty swallowing. She denies hemoptysis, GI, or vaginal bleeding. She claims her diet is good, but her appetite has varied. On physical examination, no significant physical findings are noted other than pallor. Occult blood is negative. A hemoglobin and hematocrit are ordered; the results are 7.2 g/dL and 22%, respectively. She is diagnosed with iron deficiency anemia. The results of a barium esophagram reveal esophageal webbing. Question What is the most likely diagnosis?

Plummer-Vinson syndrome Classic triad of dysphagia, iron-deficiency anemia and esophageal webs. Even though the syndrome is very rare nowadays, its recognition is important because it identifies a group of patients at increased risk of squamous cell carcinoma of the pharynx and the esophagus.

A 60-year-old man presents with headaches, weakness, and vertigo. He tells you that he has had some shortness of breath as of late. He is a non-smoker with normal BMI. The man appears flushed. Physical examination reveals clear lungs and good heart sounds, but the patient's spleen is markedly enlarged. You order a complete blood count, which includes the following information: Labs: RBC 7.2 HGB 18.3 HCT 58.2 WBC 14.1 PLT 550 A chest X-ray is normal. Question What is the most likely diagnosis? What mutation does the patient most likely have?

Polycythemia vera- overproduction of all three myeloid stem cell lines Jak2 mutation Normal values men: RBC count- 4.5-6 HGB: 13.5-17.5 HCT: 41-50% WBC: 4.5-11 PLT: 150-450

A 55-year-old man presents with chills, and he is noted to have a rise in temperature from 98.9 to 101.7° F, within 15 minutes of starting a transfusion of packed red blood cells. The transfusion is stopped; antipyretics are administered, lowering his temperature. Specimens sent to the blood bank show no evidence of hemolysis, and there are no clerical discrepancies. Question What is the most likely cause of his reaction?

Recipient antibodies against donor white cell or platelet antigens

A 40-year-old woman, undergoing a gynecologic workup for metromenorrhagia, presents with a several-week history of fatigue and lightheadedness. Laboratory evaluation reveals a hemoglobin level of 11 g/dL (12 - 16 g/dL), hematocrit of 34%, MCV of 70 fL (80 - 100 fL), and MCH of 24 pg (27 - 33 pg). Further studies reveal a ferritin level of 25 ng/mL (12 - 300 ng/mL), TIBC of 500 mcg/dL (250 - 450 mcg/dL), and an iron level of 45 mcg/dL (60 - 170 mcg/dL). The patient is diagnosed with iron-deficiency anemia secondary to menstrual blood loss, and she is given a prescription for ferrous sulfate 325 mg po tid and told to follow up in 1 month. The patient presents to the emergency department several days later. Her husband states that she had a sudden onset of dyspnea at rest and chest pain earlier that evening. The patient is extremely pale and notes continued chest pain and lightheadedness. Her EKG is unremarkable and lungs are clear to auscultation. Oxygen and nitroglycerin are administered and cardiac enzymes are drawn. The patient's hematocrit is now 23% with a hemoglobin level of 7.5. Question What treatment is most appropriate for this patient at this time?

Red blood cell transfusion

A 60-year-old man presents with a 3-day history of black stool. His past medical history is significant for bilateral DVTs and a pulmonary embolism; he is on chronic anticoagulation with warfarin. His family history is significant for colon cancer in his father. He smokes 1 pack of cigarettes per day and drinks socially. His only other past medical history is recent dyspepsia, for which omeprazole 20 mg daily was started last month. Physical exam reveals BP 90/60 mm Hg and pulse 110. The patient is dizzy when he stands up, and his BP is noted to drop to 80/60 mm Hg briefly before returning to baseline. His cardiopulmonary exam is normal, as is examination of the abdomen. He is heme+ on rectal stool exam. A CBC shows a H/H of 10/30 with normal indices, and his INR is 6.2. Question What is the most appropriate next step?

Refer to the emergency department for workup and possible admission

A 20-year-old African-American man is unconscious at presentation. Blood pressure is 115/50 mm Hg; respiratory rate is 24/min, and pulse is 160/min. Body temperature is 100.9°F. There is no clinical history available. Blood is drawn for laboratory testing. The results are shown in the table. Tests Results BUN 29 mg/dl Total bilirubin 3.6 mg/dl Indirect bilirubin 3.1 mg/dl LDH 963 U Creatinine 2.4 mg/dl Urinalysis 2+protein, Acetone+, Hemoglobin+ RBC 2.1 x 1012/l Hgb 9.4 gm/dl Hct 0.28 MCV 81 fl MCHC 33% MCH 28 pg Platelet count 15 x 109/l WBC 12 x 109/l Differential Segs 38% Bands 15% Lymphs 37% Monos 10% Nucleated RBC's 3 NRBC/100 WBC Platelets Adequate Poikilocytosis 4+ Anisocytosis 3+ Target cells Occasional Spherocytes 2+ Question Based on the peripheral blood smear - which shows cresent shaped cells and the laboratory results, what is the most likely diagnosis?

Sickle cell anemia

An 11-month-old African American boy presents with an acute onset of anorexia, irritability, unexplained bruising, and jaundice. On examination, you note pale conjunctivae, icteric sclerae, and splenomegaly. Laboratory studies reveal decreased hemoglobin and hematocrit and a significantly elevated reticulocyte count. Hemoglobin electrophoresis reveals the presence of hemoglobin S. Question What is the most likely diagnosis?

Sickle cell disease

A 45-year-old man with progressive weakness and fatigue presents with fever, shaking chills, shortness of breath, sharp chest pain, and productive cough with rusty sputum, mucosal and skin bleeding, and skin rash. He spent several weeks in a nuclear power facility after an earthquake disaster. Physical examination reveals an ill-appearing patient with signs of pallor, purpura, petechiae, stomatitis, diffuse rales, and dull percussion sound over middle parts of both lungs. His temperature is 38 C; blood pressure is 100/60; and respirations are 22. You will immediately stabilize the patient and order the necessary tests. Question What is the most specific diagnostic test for the primary disorder in this patient?

Some type of tumor Tx: Bone marrow aspirate and biopsy

A 22-year-old Asian man is presenting to a hematology clinic for the first time to seek management for his alpha thalassemia (hemoglobin H disease type). He has a strong family history of the disorder and was previously being managed by a different hematologist. Today, he receives a thorough history and physical examination, as well as various blood tests. Question What physical examination finding is most likely to be present? Purpura Splenomegaly Hemarthrosis Scleral icterus

Splenomegaly= MC finding in Thalassemia -Purpura seen in bleeding disorders (ex. ITP, TTP) -Hemarthrosis seen in hemophilia - bleeding into joint space -Scleral icterus seen in hemolytic anemia, jaundice, renal failure, etc.

A 22-year-old woman is started on methimazole for the treatment of her hyperthyroidism. She has been diagnosed with Graves' disease and has been symptomatic for the last 6 months. She is otherwise healthy and does not take any other medications. You receive a call 2 weeks later from the patient; she tells you that she has had a mild sore throat for the past 2 days and has been feeling like she is coming down with the flu. She states that the symptoms of her hyperthyroidism are a little bit better. Question What should you do?

Tell her to present ASAP for total and differential blood count. WHY? - learned about this in pharm: cannot remember

A 46-year-old woman presents with generalized petechiae, left-sided weakness, headaches, mild confusion, nausea, and vomiting. She is febrile, with an oral temperature of 103° F. On exam, she appears mildly confused and has decreased motor strength in the left extremities. Petechiae are scattered over the inner thighs and upper extremities. Significant lab values are as follows: Hgb = 7.2 g/dL, Hct = 22%, WBC = 10.2 x 103/uL, platelet count = 22,000/uL. Peripheral blood smear is remarkable for the presence of schistocytes. Coagulation studies and serum chemistries are unremarkable, with the exception of elevated bilirubin and LDH levels. Question What is the most likely diagnosis? DIC TTP ITP AIHA

Thrombotic thrombocytopenic purpura

A 35-year-old African-American woman on dialysis has had a 4-month history of hemoglobin of 9 g/dL, despite erythropoietin and intravenous iron therapies. Diagnostic studies have shown the following: Transferrin saturation 30% Ferritin 400 ng/mL Parathyroid hormone level 700 pg/mL (normal for stage 5 kidney disease 150-300 pg/mL) Serum aluminum Normal Total white blood cell count 5x10 3/mm3 Platelet count 200x10 3/mL Question What therapy should be used in addition to erythropoietin and iron to treat anemia in this patient?

Vitamin D therapy

A 14-year-old girl presents with fatigue, weakness, shortness of breath, and severe pain in her legs and abdomen. The patient denies recent illness, and she is not taking any medications. On exam, her skin is pale; there are multiple ulcers on her lower legs. She has a 101°F temperature. Question This presentation is most consistent with what diagnosis?

Sickle cell anemia

A 9-year-old African American female presents to the emergency room with a 4-hour history of "strange behavior." Her stepmother states that she appears "dazed," and she refuses to walk. She and her siblings have all had "colds" with clear rhinorrhea, sore throats, and non-productive coughs. She has "no appetite," but she has been drinking fluids "fairly well." She has had low-grade fevers up to 99.0°F orally and no history of true fevers. Otherwise, she has been well. Her stepmother denies a history of significant health problems. She states that her daughter takes a daily multivitamin and daily penicillin VK tablet. Her immunizations are up to date. She has a history of normal growth and development. Family history is significant for several family members who have a history of gallstones and a cousin who receives frequent blood transfusions. On exam, patient is a well appearing with no evidence of fluctuating consciousness or cardiorespiratory distress. Vital signs are stable. Her HEENT exam is significant for clear rhinorrhea. Cardiovascular exam demonstrates a 2/6 systolic ejection murmur. Abdominal exam demonstrates a palpable spleen 4 cm below the left costal margin. Her neurologic exam is significant for unilateral, same-sided weakness of her upper and lower extremity. She is unable to move the impaired arm or leg. She demonstrates no evidence of pain with involuntary movement of any of her extremities. The remainder of her exam is unremarkable. Available laboratory studies demonstrate a WBC of 15,000 cells/mc, Hb 10 gm/dL platelet count 250,000 per μl (microliter) of blood and peripheral blood smear demonstrates many target cells and one crescent shaped cell reported. Other laboratory studies are pending at this time. The most likely underlying cause of this patient's signs and symptoms is:

Sickle cell hemoglobinopathy Evidence- daily penicillin VK (ppx for sickle cell pt), one crescent shaped "sickle" cell on smear

A 42-year-old man presents with anemia and hematochezia; he denies syncope or chest pain. The patient's past medical history is significant for hypertension and hyperlipidemia treated with lisinopril and atorvastatin. On physical examination, he is alert and appears pale. Blood pressure is 110/70 mm Hg; pulse rate is 92/min, and respiration is 16/min. 2 minutes after rising from a seated position, blood pressure is 102/60 mm Hg and pulse rate is 110/min. The lungs are clear. Abdomen is non-tender. Gross blood is observed on rectal exam. Laboratory analysis is as follows: hemoglobin 8.1 g/dL; hematocrit 25%; platelets 200 x 109/L; and INR 0.9. 2 large-bore intravenous lines are placed and 2 L of 0.9% normal saline (NS) is administered; the patient also receives 80 mg IV of pantoprazole. The patient notes 4/10 left-sided chest pain that resolves with no intervention after a few minutes. An electrocardiogram shortly thereafter is normal. Cardiac biomarkers are drawn and pending. The patient's BP and pulse have normalized. Question What is the most appropriate next step in patient management?

Transfusion of 1 unit of packed red blood cells (pRBCs)