A 58-year-old man has a hemoglobin of 20.5 g/dL, hematocrit of 62%, and splenomegaly. Serum erythropoietin level is low, arterial oxygen saturation is normal, and JAK2 V617F mutation testing is positive. Platelet count is 550,000/uL. Which complication is he most likely to develop if untreated?
- A Recurrent gram-negative sepsis
- B Arterial and venous thrombosis ✓
- C Immune-mediated hemolytic anemia
- D Progressive cerebellar ataxia
Explanation
Polycythemia vera, defined here by elevated hematocrit, low erythropoietin, and JAK2 V617F mutation, carries a major risk of thrombosis due to hyperviscosity and platelet activation, affecting both cerebral and portal venous systems; Budd-Chiari syndrome is a classic manifestation. Treatment with phlebotomy and aspirin targets exactly this risk. Sepsis, immune hemolysis, and ataxia are not recognized complications of untreated polycythemia vera.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
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