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Acquired von Willebrand's disease in the myeloproliferative syndrome
U Budde, G Schaefer, N Mueller, H Egli, J Dent, Z Ruggeri and T Zimmerman
An acquired hemorrhagic disorder developed in two 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 I. 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 five
additional patients with the myeloproliferative syndrome, but without
bleeding symptoms, large multimers of plasma vWF were diminished also.
These findings suggest that acquired von Willebrand's disease should be
considered when a bleeding diathesis develops during the course of the
myeloproliferative syndrome.
Volume 64,
Issue 5,
pp. 981-985,
11/01/1984
Copyright © 1984 by The American Society of Hematology
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