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Springer Science and Business Media LLC Annals of Hematology 105(2)
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    초록·키워드

    Human immunodeficiency virus (HIV) is a rare cause of thrombotic microangiopathies (TMA), that can present either with normal ADAMTS13 activity (referred as HIV-related TMA) or suppressed ADAMTS13 activity (referred as HIV-related acquired thrombotic thrombocytopenic purpura, aTTP). The distinct characteristics and management of these two conditions is poorly known, given their rarity and often overlapping features. Here, we report the case of a 46-year-old female patient with TTP who received a diagnosis of HIV infection at her ADAMTS13 relapse and obtained complete remission only with antiretroviral therapy (ART). Moreover, we summarize the existing evidence in the literature about clinical presentation, outcomes and treatment of HIV-related aTTP/TMA.

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