Daly A S, Xenocostas A, Lipton J H
Department of Medical Oncology and Hematology, Princess Margaret Hospital/University Health Network, University of Toronto, Toronto, Ontario, Canada.
Bone Marrow Transplant. 2002 Dec;30(11):709-15. doi: 10.1038/sj.bmt.1703710.
A syndrome of microangiopathic hemolytic anemia, renal dysfunction and neurological abnormalities was first noted in bone marrow transplant recipients 22 years ago. Now known as transplantation-associated thrombotic microangiopathy (TA-TMA) to distinguish it from other thrombotic microangiopathies, this disorder responds poorly to conventional treatments for thrombotic thrombocytopenic purpura. In this review, we discuss the incidence and risk factors for TA-TMA and describe a pathophysiologic model of the disorder based on results obtained from laboratory models of the thrombotic microangiopathies. We conclude by suggesting possible approaches to the early diagnosis and treatment of TA-TMA based on this model that may warrant testing in future clinical trials.
22年前，骨髓移植受者中首次发现了一种微血管病性溶血性贫血、肾功能不全和神经异常综合征。现在被称为移植相关血栓性微血管病（TA-TMA），以区别于其他血栓性微血管病，这种疾病对血栓性血小板减少性紫癜的传统治疗反应不佳。在这篇综述中，我们讨论了TA-TMA的发病率和危险因素，并根据血栓性微血管病实验室模型的结果描述了该疾病的病理生理模型。我们最后基于该模型提出了TA-TMA早期诊断和治疗的可能方法，这些方法可能值得在未来的临床试验中进行测试。
