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Post‐appendectomy thrombotic thrombocytopenic purpura: A case report and review of the literature
Author(s) -
Robertson Mandy D.,
Zumberg Marc
Publication year - 2007
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.20793
Subject(s) - thrombotic thrombocytopenic purpura , microangiopathic hemolytic anemia , medicine , von willebrand factor , fresh frozen plasma , hemolytic anemia , schistocyte , plasmapheresis , adamts , gastroenterology , purpura (gastropod) , platelet , surgery , immunology , antibody , ecology , thrombospondin , metalloproteinase , matrix metalloproteinase , biology
Thrombotic thrombocytopenic purpura (TTP) is a syndrome characterized by microangiopathic hemolytic anemia and thrombocytopenia with varying degrees of renal dysfunction, neurologic signs and symptoms, and fever. Evidence has supported that a large proportion of cases of acquired TTP are due to the accumulation of ultralarge von Willebrand factor (vWF) multimers due to an acquired deficiency in the vWF cleaving protease, ADAMTS‐13. TTP is rare in the post‐surgical setting but is best described after cardiothoracic and vascular surgeries. We present a case of postoperative TTP first presenting with microangiopathic hemolytic anemia and thrombocytopenia 9 days after emergent appendectomy for a ruptured appendix. ADAMTS‐13 and factor H levels returned normal and an ADAMTS‐13 inhibitor was not identified. To our knowledge, this is the first case report of postoperative TTP after an appendectomy and the first report with correlative ADAMTS‐13 data. Plasma exchange with fresh frozen plasma followed by cryopoor plasma, along with steroids resulted in eventual remission of TTP in our patient. Early postoperative diagnosis and aggressive management with consideration of initiation of plasma exchange is imperative to decrease the morbidity and morality associated with TTP. Am. J. Hematol., 2007. © 2006 Wiley‐Liss, Inc.