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June 17, 1961


JAMA. 1961;176(11):943-944. doi:10.1001/jama.1961.03040240049016

Polycythemia vera is a malady insidious in onset, remarkable in its involvement of seemingly unrelated structures, and chronic in duration.1 The pathogenesis of the disease has not been revealed. Polycythemia vera (Vaquez-Osler disease) is to be distinguished from erythremia of high altitudes and polycythemia secondary to structural changes in the cardiopulmonary-vascular system. In addition, it must also be differentiated from polycythemias associated with neoplasm and other disorders of the kidney,2 as well as tumors in other organs. In contrast to the selective stimulation of the red cell marrow in these various conditions, in polycythemia vera all elements of the marrow are hyperplastic, and there is enlargement of splenic tissue and often liver as well.

The manifestations of polycythemia vera may be divided into three phases. 1. Symptoms develop from an enlarged blood volume and increased hematocrit level; venous thrombotic episodes may occur. 2. Complications of severe arterial disease