• 作者：L. Gilardin^a ; ¹ ; S. Malak^a ; ¹ ; Y. Schoindre^b ; L. Galicier^c ; ^d ; A. Veyradier^d ; ^e ; P. Coppo^a ; ^d ; ^{paul.coppo@sat.aphp.fr}
• 关键词：Microangiopathie thrombotique ; Purpura thrombotique thrombocytopé ; nique ; Syndrome hé ; molytique et uré ; mique ; ADAMTS13 ; Virus de l&rsquo ; immunodé ; ficience humaine
• 刊名：La Revue de Medecine Interne
• 出版年：2012
• 期刊代码：237_02488663
• 类别：med
• 出版时间：May, 2012
• 卷：33
• 期：5
• 页码：259-264
• 文件大小：501 K

Human immunodeficiency virus (HIV) infection represents a risk factor for thrombotic microangiopathy. HIV-associated thrombotic microangiopathies encompass two entities with distinct pathophysiology, clinical presentation, treatment and prognosis. Thrombotic thrombocytopenic purpura associated with human immunodeficiency virus is typically characterized by a sudden onset in a patient with a moderate immune deficiency and a few events of opportunistic diseases, and a profound acquired deficiency in the von Willebrand factor cleaving protease ADAMTS13. This diagnosis requires a well-codified management including daily therapeutic plasma exchanges, a highly active antiretroviral therapy and eventually immunomodulatory drugs. The prognosis is good with a response rate and an overall survival comparable to that of HIV-negative thrombotic thrombocytopenic purpura. On the opposite, HIV-associated thrombotic microangiopathy with a progressive onset that occurs in profoundly immunocompromised patients with past history of multiple opportunistic diseases usually have a detectable ADAMTS13聽activity and a worse prognosis. Usual treatment is poorly efficient. Forthcoming studies should assess the role of immunomodulatory drugs such as rituximab in the setting of HIV-associated thrombotic microangiopathy, and identify possible risk factors associated with the occurrence of these diseases.