Premium
Postoperative thrombocytopenia in type IIB von Willebrand disease
Author(s) -
Hultin Mae B.,
Sussman Ira I.
Publication year - 1990
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830330113
Subject(s) - medicine , platelet , von willebrand factor , von willebrand disease , coagulopathy , disease , surgery , emotional stress , bleeding time , gastroenterology , platelet aggregation
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.