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[继发性血栓性微血管病]

[Secondary thrombotic microangiopathies].

作者信息

Coppo P

机构信息

Service d'hématologie, centre de référence des microangiopathies thrombotiques, hôpital Saint-Antoine, UPMC université Paris 6, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France.

Service d'hématologie, centre de référence des microangiopathies thrombotiques, hôpital Saint-Antoine, UPMC université Paris 6, 184, rue du Faubourg-Saint-Antoine, 75012 Paris, France.

出版信息

Rev Med Interne. 2017 Nov;38(11):731-736. doi: 10.1016/j.revmed.2017.06.025. Epub 2017 Sep 8.

Abstract

Thrombotic microangiopathies (TMA) are termed secondary when associated to a specific context favouring their occurrence. They encompass mainly TMA associated with pregnancy, allogeneic hematopoietic stem cell transplantation, cancer, drugs, or HIV infection. Secondary TMA represent a heterogeneous group of diseases which clinical presentation largely depends on the associated context. It is therefore mandatory to recognize these conditions since they have a significant impact in TMA management and prognosis. A successful management still represents a challenge in secondary TMA. Significant progresses have been made in the understanding of pregnancy-associated TMA, allowing an improvement of prognosis; on the opposite, other forms of secondary TMA such as hematopoietic stem cell transplantation-associated TMA or TMA associated with chemotherapy remain of dismal prognosis. A better understanding of pathophysiology in these forms of TMA, in association with a more empirical approach through the use of new therapeutic agents that can also help in the understanding on new mechanisms a posteriori, should improve their prognosis. The preliminary encouraging results reported with complement blockers in this field could represent a convincing example.

摘要

血栓性微血管病(TMA)在与有利于其发生的特定背景相关联时被称为继发性。它们主要包括与妊娠、同种异体造血干细胞移植、癌症、药物或HIV感染相关的TMA。继发性TMA代表一组异质性疾病,其临床表现很大程度上取决于相关背景。因此,认识到这些情况是必不可少的,因为它们对TMA的管理和预后有重大影响。在继发性TMA中,成功的管理仍然是一项挑战。在理解妊娠相关TMA方面已经取得了重大进展,从而改善了预后;相反,其他形式的继发性TMA,如造血干细胞移植相关TMA或与化疗相关的TMA,预后仍然很差。更好地理解这些形式的TMA的病理生理学,结合通过使用新的治疗药物采取更经验性的方法,这也有助于事后理解新机制,应该可以改善它们的预后。在这一领域使用补体阻滞剂报告的初步令人鼓舞的结果可能就是一个有说服力的例子。

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