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TO THE EDITOR: Polycythemia vera (PV) is a chronic myeloproliferative disorder with panmyelosis that is unrelated to a secondary cause. Although the discovery of JAK2 V617F mutation in the pathogenetic mechanism of PV has made the diagnosis clearer and easier, until recently, diagnosis of PV involved excluding secondary causes of erythrocytosis in clinical practice. Among the secondary causes, smoking history, elevated erythropoietin (EPO) level, and hypoxemia measured by arterial blood gas analysis are well known and recognized as minor criteria for the diagnosis of PV. With the upfront evaluation of JAK2 mutation status in patients with erythropoiesis, clinicians are apt to be neglectful about the evaluation of these secondary causes. However, based on our case, evaluation of other causes is clinically significant.

JAK2V617F-positive polycythemia vera accompanied by renal artery stenosis