title: [Human immunodeficiency virus-associated thrombotic microangiopathies].
abstract: 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 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.
authors.affiliation: Département d'hématologie, UPMC université Paris-06, hôpital Saint-Antoine, 184, rue du Faubourg-Saint-Antoine, AP-HP, 75012 Paris, France.
authors.forename: L
authors.initials: L
authors.lastname: Gilardin
authors.forename: S
authors.initials: S
authors.lastname: Malak
authors.forename: Y
authors.initials: Y
authors.lastname: Schoindre
authors.forename: L
authors.initials: L
authors.lastname: Galicier
authors.forename: A
authors.initials: A
authors.lastname: Veyradier
authors.forename: P
authors.initials: P
authors.lastname: Coppo
mesh_terms: D015658:HIV Infections / Q000150:complications*; D006801:Humans; D011379:Prognosis; D057049:Thrombotic Microangiopathies / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy