PANRE Hematology 2

Question 1

A 55-year-old male presents with plethora, headache, and pruritus after bathing.
Hgb is 19.4 g/dL, Hct 58%, WBC 13,000/μL, platelets 520,000/μL.
JAK2 V617F mutation is positive.
What is the most likely diagnosis?

Accepted Answer

Polycythemia vera

Answer

Secondary polycythemia

Answer

Essential thrombocythemia

Answer

Chronic myelogenous leukemia

Question 2

A 24-year-old African American male develops hemolytic anemia and hemoglobinuria after being prescribed dapsone for dermatitis herpetiformis. Peripheral smear shows Heinz bodies. What is the underlying defect?

Accepted Answer

Glucose-6-phosphate dehydrogenase (G6PD) deficiency

Answer

Pyruvate kinase deficiency

Answer

Hereditary spherocytosis

Answer

Sickle cell trait

Question 3

A 6-year-old boy has recurrent hemarthroses and prolonged bleeding after minor trauma. PTT is 68 seconds (normal <35), PT is normal, platelet count is normal, bleeding time is normal. Factor VIII activity is 2%. What is the diagnosis?

Accepted Answer

Hemophilia A

Answer

Hemophilia B

Answer

Von Willebrand disease type 3

Answer

Factor VII deficiency

Question 4

A 30-year-old presents with pancytopenia: Hgb 7.1, WBC 1,800, platelets 28,000. Bone marrow biopsy shows a hypocellular marrow with >70% fat cells. No splenomegaly. What is the most appropriate treatment for severe aplastic anemia in a patient with a matched sibling donor?

Accepted Answer

Allogeneic hematopoietic stem cell transplantation

Answer

Immunosuppression with antithymocyte globulin and cyclosporine

Answer

G-CSF therapy alone

Answer

Corticosteroids and erythropoietin

Question 5

A patient has a positive direct Coombs test, elevated indirect bilirubin, low haptoglobin, and reticulocytosis. Peripheral smear shows spherocytes. Splenomegaly is present. What condition is most consistent with these findings?

Accepted Answer

Autoimmune hemolytic anemia

Answer

Hereditary spherocytosis

Answer

Microangiopathic hemolytic anemia

Answer

Paroxysmal nocturnal hemoglobinuria

Question 6

A post-surgical ICU patient develops DIC. Labs show: PT prolonged, PTT prolonged, fibrinogen 80 mg/dL, D-dimer markedly elevated, platelet count 42,000/μL, and schistocytes on smear. What is the most critical initial management step?

Accepted Answer

Treat the underlying cause and replace clotting factors with FFP and cryoprecipitate

Answer

Administer heparin to prevent further clotting

Answer

Platelet transfusion alone

Answer

Administer tranexamic acid immediately

Question 7

A patient receiving unfractionated heparin for 8 days develops a platelet count drop from 230,000 to 65,000/μL and a new arterial thrombosis. What is the most appropriate next step?

Accepted Answer

Discontinue heparin and start a direct thrombin inhibitor such as argatroban

Answer

Increase the heparin dose to achieve therapeutic anticoagulation

Answer

Switch to low-molecular-weight heparin

Answer

Administer platelet transfusion