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To describe and analyze the clinical and biological manifestations of thrombotic microangiopathy (TMA) and evaluate the therapeutic strategies.A retrospective study was performed in 14 infectious diseases units of southern France. All HIV patients presenting TMA during the last decade were selected (n = 14).The principle signs observed, aside from defining TMA (association of microangiopathic hemolytic anemia, thrombocytopenia and renal failure), were diarrhea (n = 9) and a high level of lacticodehydrogenase (LDH). Patients were at an advanced stage of HIV infection (median of CD4+ cells counts = 70/mm3). Specific etiology was identified in 7 cases: bacterial infection (n = 5), CMV retinitis associated with Kaposi's sarcoma (n = 1) and intravenous drug use (n = 1). Various treatments were used: plasmatic exchange (5 cases), fresh frozen plasma (6 cases), steroid therapy (4 cases), acetyl salicylic acid (3 cases), adaptation of antiretroviral treatment (3 cases). The global prognostic was poor with 50% mortality within 2 months. A high level of LDH (2500 UI/L) was associated with premature death in 5 of the 6 cases observed.Our data confirm the severity of TMA in case of HIV infection. The common treatment remains the use of plasmatic exchange and fresh frozen plasma; an optimal antiretroviral therapy must be associated.