ACQUIRED VONWILLEBRANDS DISEASE IN THE MYELOPROLIFERATIVE SYNDROME

  • 1 January 1984
    • journal article
    • research article
    • Vol. 64  (5) , 981-985
Abstract
An acquired hemorrhagic disorder developed in 2 patients in association with postsplenectomy thrombocytosis and leukocytosis during the course of the myeloproliferative syndrome. The presence of acquired von Willebrand''s disease in these individuals was demonstrated by a decrease or absence of the larger von Willebrand factor (vWF) multimers, alteration of the repeating vWF multimeric triplet, decreased ristocetin cofactor activity (vWF:RCo), and prolonged bleeding time. The bleeding stopped in both patients after treatment with either 1-deamino-[8-D-arginine]-vasopressin (DDAVP) or Cohn fraction 1. Treatment with thrombocytapheresis and azathioprine or busulfan resulted in reduction of the elevated platelet and white cell counts and was associated with partial correction of the vWF abnormalities and remission of the hemostatic abnormalities. In 5 additional patients with the myeloproliferative syndrome, but without bleeding symptoms, large multimers of plasma vWF were diminished also. Acquired von Willebrand''s disease should be considered when a bleeding diathesis develops during the course of the myeloproliferative syndrome.