Incidence And Clinical Presentation

The incidence of polycythemia vera (PV) is slightly higher in men than in women (2.8 vs 1.3 cases per 100,000 per year).1 The mean age at diagnosis is 62 years (range, 20- 85).2

The clinical presentation of patients with PV can be nonspecific, and may include headaches (48%), weakness (47%), dizziness (43%,) pruritus (43%), and excessive sweating (33%). Most patients are asymptomatic and come to a hematologist's attention based on a "routine" complete blood count (CBC).

Erythromelalgia (burning pain in the feet and hands accompanied by erythema, pallor, or cyanosis) is common in PV and is considered to be secondary to microvascular thrombotic complications (also seen in essential thrombocythemia, ET). It is more common when platelet counts are above 400,000/ ^L.3

Venous and arterial thrombosis are also common in PV patients and are related to increased blood viscosity and other unknown factors. Major thrombotic events can happen in 15% of the patients and include cerebrovascular accidents, myocardial infarction, superficial thrombophlebitis, deep venous thrombosis, pulmonary emboli, Budd-Chiari syndrome, and mesenteric throm-bosis.4

Gastrointestinal symptoms of peptic ulcer disease are common and can be attributed to histamine release from tissue basophils and from Helicobacter pylori.5

On physical exam, patients often will have splenomegaly (70%), facial plethora (67%), and hepatomegaly (40%).

Laboratory abnormalities may include elevated hematocrit and red blood cell mass in almost all patients, platelet count >400,000/^L (in 60%), and a white blood cell (WBC) count >12,000/^L (in 40%). Bone marrow cellularity is increased in 90% of cases, and iron storage is absent in 95%.

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