The association of polycythemia with a cerebellar hemangioblastoma: The production of an erythropoiesis stimulating factor by the tumor

Abstract

A patient with associated cerebellar hemangioblastoma and polycythemia is presented. The circulating red cell volume was increased to 44 cc. per kg., the plasma volume was within normal limits. The apparent Gr 51 red cell life span ( T 1 2 ) was twenty-six days. The plasma iron turnover and red cell iron turnover were accelerated to 0.47 and 0.44 mg. per kg. per day, respectively, indicating increased red cell synthesis. There was normal incorporation and release of Fe 59 over the sacral marrow on body surface counting indicating red cell synthesis, but no production of red cells by the cerebellar tumor or other extramedullary sites. Fluid aspirated from the cystic cerebellar tumor of this patient was shown to contain erythropoiesis stimulating activity when compared to normal plasma or spinal fluid. No significant increase of erythropoiesis stimulating activity was detected in the urine, plasma or spinal fluid of the patient. The active substance in the tumor fluid appears to be a non-dialyzable, partially heat-denaturable substance. It is suggested that the polycythemia occurring in association with cerebellar hemangioblastomas is caused by increased production of red blood cells by the marrow in response to an erythropoiesis stimulating factor produced by the tumor.