[Polycythemia vera: clinical aspects and course in 86 patients]

We analyze symptoms, clinical course, and survival time of 86 patients with polycythemia vera treated between 1966 and 1987 at the medical polyclinic of the University Hospital of Zürich. The mean age of disease onset in 40 men and 46 women studied was 59 years. Most commonly the first symptoms were vertigo and headache (49%) and pain in the extremities (42%). Clinically, plethora was found in half of the cases and 56% showed signs of abnormal arterial and venous circulation. Two thirds of the patients had thromboembolic complications and 40% had hemorrhages chiefly occurring in the gastrointestinal tract. 48% of the patients died after an average survival time of 10 years. The most common cause of death (46%) was acute myelogenous leukemia, followed in 32% by thrombosis and/or embolism and in 7% by death due to hemorrhage. 18 of the 19 leukemia patients were treated with myelosuppressive agents. Patients treated with 32P showed a substantially higher incidence of malignancy than the group of patients not receiving 32P (p less than 0.001). The development of malignancies also seems to be related to the dosage of 32P. Patients who developed malignancies generally received higher doses of 32P (30 vs 20 mCi). Therefore, consistent phlebotomy therapy and restrictive chemotherapy combined with low dosage salicylates appears to be superior to 32P-therapy.