Primary hemorrhagic thrombocythemia

Abstract

1. 1. Six new cases of primary hemorrhagic thrombocythemia are reported. All six patients exhibited thrombohemorrhagic phenomena in one form or another. Purpura, epistaxis, and bleeding of the gums and gastrointestinal tract were common. All had hepatosplenomegaly. In the patient with gastrointestinal bleeding, sources could not be found. All had persistent elevation of thrombocyte count above 800,000/ cu. mm. (indirect method). Acute myeloblastic leukemia finally developed in one patient. 2. 2. The literature is critically surveyed. On the basis of this survey and from personal experience new diagnostic criteria are outlined. These include: (a) a history of thrombohemorrhagic phenomena; (b) the presence of the spleen, which is usually enlarged; (c) erythrocyte level of not more than 6 million/cu. mm., hemoglobin not to exceed 18 gm. per cent, hematocrit not above 54 per cent, leukocytes not exceeding 50,000/cu. mm., and a platelet count persistently above 800,000/cu. mm. (indirect method); (d) hyperplasia of all marrow elements with megakaryocytic preponderance and platelet masses plus eosinophilia and/or basophilia; and (e) absence of leukemic tissue infiltration. 3. 3. It is emphasized that primary hemorrhagic thrombocythemia is a myeloproliferative syndrome in which elevation of platelets predominates, thus constituting a separate entity apart from polycythemia vera, chronic granulocytic leukemia and secondary thrombocytoses. 4. 4. The recognition of this entity is clinically important in guiding management. Radioactive phosphorus appears to be the treatment of choice at the present time.