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Polycythemia vera — ABIM Board MCQ

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ModerateHematology/OncologyPolycythemia veraABIM Board

A 62-year-old has aquagenic pruritus, erythromelalgia, hemoglobin 19.2 g/dL, hematocrit 58%, leukocytosis, thrombocytosis, a subnormal erythropoietin level and a JAK2 V617F mutation. Oxygen saturation is normal. What is the most likely diagnosis?

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Correct answer: A — JAK2-positive polycythemia vera with panmyelosis

The best answer is “JAK2-positive polycythemia vera with panmyelosis”. Marked sustained erythrocytosis with panmyeloid count elevation, low erythropoietin and a JAK2 driver mutation is the characteristic polycythemia vera phenotype. Hypoxemic secondary erythrocytosis usually raises erythropoietin, while volume contraction does not explain leukocytosis, thrombocytosis or the molecular finding. Essential thrombocythemia lacks this dominant erythrocytosis, and chronic myeloid leukemia is defined by BCR-ABL1 rather than JAK2.

Reference: International Consensus Classification Criteria for Polycythemia Vera: https://onlinelibrary.wiley.com/doi/full/10.1002/ajh.27002