Mehta Neha, Saxena Ashish, Niesvizky Ruben
Department of Medicine, New York Presbyterian-Weill Cornell Medical Center, New York, NY, USA.
BMJ Case Rep. 2012 Aug 1;2012:bcr2012006461. doi: 10.1136/bcr-2012-006461.
Thrombotic thrombocytopaenic purpura (TTP) is a thrombotic microangiopathy characterised by haemolytic anaemia, schistocytes on peripheral blood smear and thrombocytopaenia that can additionally present with fever, renal insufficiency and/or neurological abnormalities. While our understanding of idiopathic TTP has significantly advanced, there are still many unanswered questions regarding the pathophysiology of secondary TTP, which can be associated with malignancy, pregnancy, HIV infection, bone marrow transplantation and exposure to certain drugs including several chemotherapeutic agents. Here we present a case of bortezomib-associated TTP. Our patient developed microangiopathic haemolytic anaemia, thrombocytopaenia and altered mental status after her ninth dose of bortezomib. The drug was subsequently discontinued and the patient underwent one session of plasma exchange with clinical improvement and recovery of her platelet counts, disappearance of schistocytes and resolution of both neurological symptoms and haemolysis in 2 days.
血栓性血小板减少性紫癜(TTP)是一种血栓性微血管病,其特征为溶血性贫血、外周血涂片可见裂体细胞以及血小板减少,此外还可伴有发热、肾功能不全和/或神经功能异常。虽然我们对特发性TTP的认识有了显著进展,但关于继发性TTP的病理生理学仍有许多问题未得到解答,继发性TTP可能与恶性肿瘤、妊娠、HIV感染、骨髓移植以及接触某些药物(包括几种化疗药物)有关。在此我们报告一例硼替佐米相关的TTP病例。我们的患者在接受第九剂硼替佐米后出现微血管病性溶血性贫血、血小板减少和精神状态改变。随后停用了该药物,患者接受了一次血浆置换,临床症状改善,血小板计数恢复,裂体细胞消失,神经症状和溶血在2天内均得到缓解。