Post‐appendectomy thrombotic thrombocytopenic purpura: A case report and review of the literature

Abstract

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. 82:224-228, 2007. V V C 2006 Wiley-Liss, Inc.

Survival and relapse in patients with thrombotic thrombocytopenic purpura

“…[2][3][4] Patients defined as idiopathic were also heterogeneous. Although these patients were not recognized to have any of the conditions defining the other established clinical categories, some had preceding or concurrent conditions, such as pancreatitis, 34 infections, 35 or surgery, 36,37 which may have triggered the onset of TTP, reflecting the current arbitrary definition of "idiopathic" TTP. Only 46 (47%) of the 98 patients defined as idiopathic had ADAMTS13 activity Ͻ 10%.…”

Atypical presentations of thrombotic thrombocytopenic purpura: A review

“…Most of these case reports are described in the pre-ADAMTS13 era. Therefore, there is not much data on ADAMTS13 deficiency and postsurgical TTP except for recent ones where enzyme deficiency was identified in one case [28] but not the other [29]. Generally, there is no relapse of TTP once the postsurgical event is treated.…”

Thrombotic microangiopathic syndromes associated with drugs, HIV infection, hematopoietic stem cell transplantation and cancer

“…HIV infection may be a coincidental diagnosis in patients with TTP or it may provide an inflammatory stimulus for initiating an acute episode of TTP in susceptible subjects, similar to reports of TTP associated with infections [60], surgery [61], pancreatitis [62], and pregnancy [63]. In most patients with HIV infection in whom the diagnosis of TTP is considered, an alternative explanation for the thrombocytopenia and microangiopathic hemolytic anemia will be discovered.…”