Hematology: Challenging Cases with Your Participation COPYRIGHT

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1 Hematology: Challenging Cases with Your Participation Reed E. Drews, MD Beth Israel Deaconess Medical Center Harvard Medical School Boston, MA

2 Question 1

3 Question 1 64-year-old man is evaluated during routine exam He takes aspirin and acetaminophen for osteoarthritis On exam, pallor is absent. BP = 116/72 with no orthostatic changes; P = 68.

4 Labs Hgb = 9.7 g/dl WBC = 5.8 K/μL MCV = 83 fl Plts = 265 K/μL Retic count = 0.5% LDH = 80 U/L Iron = 40 μg/dl TIBC = 200 μg/dl Ferritin = 210 ng/ml Peripheral smear is normal

5 Which of the following is the most likely diagnosis? A. Inflammatory anemia B. Hemoglobin C disease C. Iron deficiency D. Thalassemia

6 Question 2

7 Question 2 27-year-old woman undergoes follow-up evaluation 5 months after diagnosis of unprovoked PE for which she was prescribed a 6-month course of anticoagulant therapy Family history Maternal grandmother took warfarin for many years for unknown reason Older brother with DVT at age 32 years Meds: no contraceptives or other medications Labs: CBC normal; INR = 3.0

8 Which of the following is the most appropriate next step in the evaluation of this patient? A. Immediate thrombophilic screening B. JAK2 mutation analysis C. No further evaluation needed D. Thrombophilic screening at least 2 weeks after therapy cessation

9 T H G I R Question Y P O C 3

10 Question 3 A 68-year old woman has hypertension for which she takes HCTZ and low-dose aspirin daily. On routine blood testing, she is found to have a high total protein. On exam, she appears well without lymphadenopathy, hepatosplenomegaly or bony pain to palpation or percussion. Hgb = 12.9 g/dl; WBC = 6.2K/μL; platelets = 245K/μL. SPEP shows 1.2 g/dl monoclonal IgG-kappa. UPEP is negative for protein, including Bence Jones protein.

11 Question 3 (continued) Bone marrow biopsy shows 7% plasma cells, and skeletal survey is normal, without lytic lesions. Which is the most appropriate management for this patient? 1. Obtain a bone scan 2. Begin bisphosphonate therapy 3. Begin thalidomide + dexamethasone 4. Monitor expectantly and repeat the SPEP and UPEP in 3 to 6 months

12 Question 4

13 Question 4 A 65-year old presents with cervical adenopathy and splenomegaly. HCT = 37%; WBC = 34,000/µL with 2% polys and 98% lymphs; platelets = 177,000/µL. Peripheral blood smear review reveals mature appearing lymphocytes and smudge cells (see next slide).

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15 Question 4 (continued) Which of the following is true of this patient? 1. Patients in late childhood or young adulthood are as likely to have this disease as older patients 2. One should be alert for the blast phase of disease 3. The patient might present with autoimmune hemolytic anemia and/or immune thrombocytopenia 4. The evidence favors early treatment

16 Question 5

17 Question 5 A 63-year old patient develops exertional dyspnea. On exam, sclerae are slightly icteric. HCT = 28% with MCV = 105; reticulocyte count = 9%; WBC and platelet counts are normal. The laboratory reports bite cells on peripheral blood smear (see next slide).

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19 Question 5 (continued) What is the most likely underlying mechanism of anemia in this patient? 1. Autoimmune hemolysis 2. Microangiopathic hemolytic anemia 3. Oxidant hemolysis 4. Paroxysmal nocturnal hemoglobinuria

20 Question 6

21 Question 6 A 24-year old patient develops dyspnea and is found to be anemic with HCT = 28%, MCV = 104 fl, and reticulocyte count = 6%. The laboratory reports 2+ spherocytes on peripheral blood smear (see next slide).

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23 Question 6 (continued) Coombs testing is positive for IgG and negative for C3. What is the most appropriate management in this patient? 1. Transfuse packed red blood cells 2. Begin prednisone 1 mg/kg and folic acid 5 mg daily 3. Begin danazol 4. Administer rituximab

24 The End