Regner Sean R, Lerfald Nathan
Department of Medicine, West Virginia University Hospital, Morgantown, West Virginia.
J Clin Apher. 2019 Aug;34(4):510-512. doi: 10.1002/jca.21687. Epub 2019 Feb 18.
Thrombotic microangiopathies are rare diseases associated with significant morbidity and mortality. The treatment of thrombotic thrombocytopenic purpura (TTP) and drug-induced thrombotic microangiopathy (DITMA) remains a diagnostic dilemma as they present similarly but respond differently to standard treatment with plasma exchange. TTP is a deficiency of a disintegrin and metalloprotease with thrombospondin type 1 motif 13 resulting in von Willebrand factor aggregates. DITMA is due to vascular and platelet toxicity. Our case contradicts a notion in current literature that suggests supportive therapy when there is high suspicion for DITMA. We present what appears to be the second published case of cocaine temporally associated with TTP. Our case responded to therapy. We propose this case should influence weighing the risks and benefits of treatment of suspected DITMA and reinforces current official guidelines that suggest treating cases of suspected DITMA as TTP until the diagnosis is confirmed.
血栓性微血管病是一类罕见疾病,与显著的发病率和死亡率相关。血栓性血小板减少性紫癜(TTP)和药物性血栓性微血管病(DITMA)的治疗仍然是一个诊断难题,因为它们临床表现相似,但对血浆置换的标准治疗反应不同。TTP是一种具有血小板反应蛋白1型基序的去整合素和金属蛋白酶13缺乏症,导致血管性血友病因子聚集。DITMA是由于血管和血小板毒性所致。我们的病例与当前文献中的一种观点相矛盾,该观点认为在高度怀疑DITMA时应采取支持性治疗。我们报告了似乎是第二例在时间上与TTP相关的可卡因病例。我们的病例对治疗有反应。我们建议该病例应影响对疑似DITMA治疗的风险和益处的权衡,并强化当前的官方指南,该指南建议在确诊之前,将疑似DITMA病例按TTP进行治疗。