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Postoperative thrombocytopenia in type IIB von Willebrand disease

✍ Scribed by Dr. Mae B. Hultin; Ira I. Sussman


Publisher
John Wiley and Sons
Year
1990
Tongue
English
Weight
572 KB
Volume
33
Category
Article
ISSN
0361-8609

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✦ Synopsis


Abstract

We report studies of a large kindred with type IIb von Willebrand disease and manifestations of thrombocytopenia. While only one member of the family was thrombocytopenic routinely, three members of the family who underwent various surgical procedures demonstrated thrombocytopenia and platelet clumping postoperatively. Platelet clumps were found on peripheral blood smear only in the immediate postoperative specimens and did not appear to be a technical artifact. In the one patient who received no preoperative prophylactic therapy, postoperative plasma specimens showed the transient appearance of high molecular weight von Willebrand factor multimers. These results support the hypothesis that surgery, or some related aspect such as stress, led to the release of high molecular weight multimers, resulting in platelet clumping and removal from the circulation, and subsequent thrombocytopenia. Thrombocytopenia under conditions of stress may be a more common manifestation of type IIb vWd than is currently appreciated.


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