ANEMIA DUE TO STABLE FACTOR DEFICIENCY IN A PATIENT WITH POLYCYTHEMIA VERA

Publisher Website
Google Scholar
Abstract
Hemorrhagic tendency in polycythemia vera has been reported in the literature. However, bleeding severe enough to cause anemia and to mask the diagnosis of polycythemia vera is rare. A 23-year-old man developed splenomegaly in 1948. He was admitted several times to the hospital for gastrointestinal hemorrhage during the years 1956 to 1959. In 1956 splenectomy was performed. The spleen weighed 887 gm and showed myeloid metaplasia. Several X-ray studies failed to reveal any evidence of peptic ulcer or esophageal varices. Because of severe anemia and splenomegaly, a diagnosis of agnogenic myeloid metaplasia was made. The bone marrow on several occasions was hyperplastic. The prothrombin time was increased on several occasions and a deficiency of stable factor was established. The patient was treated with iron for hemorrhagic anemia following which he developed erythrocytosis, thrombocytosis, leucocytosis and plethoric appearance. A diagnosis of polycythemia vera with stable factor deficiency was finally made. During polycythemia phase the patient developed priapism, a rare thrombotic complication of this disease. This necessitated amputation of his penis. The patient was treated with myeleran for polycythemia vera which resulted in good hematologic response. The prothrombin time became normal after treatment. The stable factor deficiency is believed to be acquired in type since no history of bleeding manifestation could be obtained in other members of his family and the patient himself had no bleeding episodes, prior to age 23.

This publication has 6 references indexed in Scilit: