Blood Transfusion Unit, Kansai Medical University Takii Hospital, Moriguchi, Japan.
Int J Gen Med. 2012;5:307-11. doi: 10.2147/IJGM.S30271. Epub 2012 Mar 28.
Thrombotic thrombocytopenic purpura (TTP) is a multisystemic microvascular disorder that may be caused by an imbalance between unusually large von Willebrand factor multimers and the cleaving protease ADAMTS13. In acquired TTP, especially in secondary TTP with various underlying diseases, the diagnosis is difficult because there are many cases that do not exhibit severe deficiency of ADAMTS13 or raised levels of ADAMST13 inhibitors. It is well known that collagen disease, malignancy, and hematopoietic stem cell transplantation can be underlying conditions that induce TTP. However, TTP induced by acute pancreatitis, as experienced by our patient, has rarely been reported. Our patient completely recovered with treatments using steroids and plasma exchange (PE) only. In cases where patients develop acute pancreatitis with no apparent causes for hemolytic anemia and thrombocytopenia, the possibility of TTP should be considered. Treatments for TTP including PE should be evaluated as soon as a diagnosis is made.
血栓性血小板减少性紫癜(TTP)是一种多系统的微血管疾病,可能是由于超大 von Willebrand 因子多聚体与切割蛋白酶 ADAMTS13 之间的失衡引起的。在获得性 TTP 中,尤其是在伴有各种基础疾病的继发性 TTP 中,由于许多病例并未表现出 ADAMTS13 的严重缺乏或 ADAMTS13 抑制剂水平升高,因此诊断较为困难。众所周知,胶原病、恶性肿瘤和造血干细胞移植都可能是导致 TTP 的基础疾病。然而,由急性胰腺炎引起的 TTP ,如我们的患者所经历的那样,很少有报道。我们的患者仅通过使用类固醇和血浆置换(PE)治疗就完全康复了。在那些发生急性胰腺炎而无明显溶血性贫血和血小板减少原因的患者中,应考虑 TTP 的可能性。一旦确诊,应评估包括 PE 在内的 TTP 治疗方法。