Kheder El-Fekih Rania, Deltombe Clément, Izzedine Hassan
Service de néphrologie, hôpital de La Rabta, La Rabta, 1007 Tunis, Tunisie.
Service de néphrologie, immunologie clinique, transplantation, CHU Hôtel-Dieu, Place Alexis-Ricordeau, 44000 Nantes, France.
Nephrol Ther. 2017 Nov;13(6):439-447. doi: 10.1016/j.nephro.2017.01.023. Epub 2017 Jul 31.
Thrombotic microangiopathy (TMA) is a group of disorders characterized by mechanical hemolytic anemia with thrombocytopenia and an ischemic organic lesion of variable and potentially fatal importance affecting mostly the kidneys and the brain with histologically a disseminated and occlusive microvasculopathy. The incidence of TMA represents 15% of acute kidney failure in oncological setting, largely due to the introduction of anti-angiogenic agents over the past decade. It may be more rarely related to cancer itself. The iatrogenic TMA can be classified into 2 types: The type I, secondary to chemotherapy (mitomycinC, gemcitabine), exposes to a chronic dose-dependent renal injury as well as an increase in morbidity and mortality; iatrogenic type II, secondary to anti-angiogenic agents', results in a dose-independent renal involvement and renal functional recovery is usual when the drug is discontinued. There is no randomized controlled trial to establish EBM-type management in TMA support. However, complement activation pathways and regulatory factors analyses allowed us to understand the mechanisms of endothelial lesions. As a result, the current trend includes the use of immunosuppressive agents in recurrent or plasmapheresis-refractory MAT.
血栓性微血管病(TMA)是一组以机械性溶血性贫血伴血小板减少以及具有不同程度且可能危及生命的缺血性器质性病变为特征的疾病,主要影响肾脏和大脑,组织学上表现为弥漫性和闭塞性微血管病。在肿瘤学背景下,TMA的发病率占急性肾衰竭的15%,这在很大程度上归因于过去十年中抗血管生成药物的引入。它可能很少与癌症本身相关。医源性TMA可分为2种类型:I型继发于化疗(丝裂霉素C、吉西他滨),会导致慢性剂量依赖性肾损伤以及发病率和死亡率增加;医源性II型继发于抗血管生成药物,导致剂量非依赖性肾受累,停药后肾功能通常会恢复。目前尚无随机对照试验来确立TMA支持治疗中循证医学类型的管理方法。然而,补体激活途径和调节因子分析使我们能够了解内皮损伤的机制。因此,当前的趋势包括在复发性或血浆置换难治性TMA中使用免疫抑制剂。