title: Factor V deficiency in Philadelphia-positive chronic myelogenous leukemia.
abstract: Factor V deficiency has been identified in 8 of 8 patients 7--20 yr of age, with Philadelphia-positive (Ph1+) chronic myelogenous leukemia (CML). In these 8 patients, factor V deficiency was not due to hepatic dysfunction, factor V inhibitors, or disseminated intravascular coagulation. In 3 patients, factor V activity rose 10%--12% (0.10--0.12 U/ml) after the infusion of 28--31 ml/kg body weight of fresh frozen plasma (FFP). The rise persisted less than 14 hr. The mean measured postinfusion rise in factor V was 18% of the expected rise calculated from the volume of FFP infused in the patients' plasma volume. In 4 patients, a small transient rise in factor V activity occurred after splenectomy or plateletpheresis. Factor V deficiency was completely corrected after a marked reduction in bone marrow cellularity in 2 patients with Ph1+ CML treated with extensive chemotherapy, total body irradiation, and bone marrow transplantation. Factor V deficiency was retrospectively observed in 6 of 20 patients, ages 20--80 yr, with Ph1+ CML and 3 of 6 patients with other myeloproliferative disorders. The factor V deficiency appears to be associated with the large myeloid-megakaryocytic cell mass characteristic of CML and other myeloproliferative disorders.
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001780:Blood Coagulation Tests; D001803:Blood Transfusion; D016026:Bone Marrow Transplantation; D002648:Child; D002904:Chromosomes, Human, 21-22 and Y; D002908:Chronic Disease; D004211:Disseminated Intravascular Coagulation; D005165:Factor V / Q000037:antagonists & inhibitors / Q000378:metabolism; D005166:Factor V Deficiency / Q000150:complications* / Q000628:therapy; D006801:Humans; D006918:Hydroxyurea / Q000494:pharmacology; D007951:Leukemia, Myeloid / Q000097:blood / Q000150:complications / Q000235:genetics*; D008111:Liver Function Tests; D008875:Middle Aged; D017713:Platelet Transfusion
chemical_list: D005165:Factor V; D006918:Hydroxyurea