Thrombotic microangiopathy in HIV-infected patients. Review of literature and case report

Thrombotic microangiopathy (TMA) is a syndrome representing a broad range of diseases and conditions, and characterized by microvasculature occlusion, thrombocytopenia, microangiopathic hemolytic anemia, and ischemic organ damage. Currently, classification distinguishes two primary forms of TMA - thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS). Various secondary TMA forms are associated with pregnancy, organ transplantation, infections, autoimmune diseases, malignancies, drug exposure, and other conditions. The key feature of TTP pathogenesis is decreased activity of ADAMTS-13 metalloprotease, the classical clinical presentation is a tirade of microangiopathic hemolytic anemia, severe thrombocytopenia, and central nervous system damage. TTP may be innate or immune-mediated; the latter may be idiopathic or associated with underlying conditions, again like autoimmune diseases, drugs, pregnancy, malignancies, and infections. TMA associated with HIV-infection was described in 1984, and since that time, many cases and case series had been reported. Even though TMA is a relatively rare complication of HIV-infection, nowadays association between these conditions is well established. Endothelial damage, which is now considered a primary cause of platelet activation and microvasculature thrombosis, in HIV-infection may be a consequence of the direct viral invasion, or indirectly associated with cytokines and HIV-related proteins. HIV poses a risk of TTP 15-40 times higher compared to the non-infected persons, and the incidence of HIV-associated HUS is even higher. However, regardless of its form, TMA in HIV-infected patients known to be associated with 6-fold increased mortality. Although the majority of patients with HIV-associated TMA present with late stages of severe HIV-infection, in 28% of cases TMA turns to be the first presentation of the disease. We present here a case of immune-mediated TTP, associated with HIV-infection. In this particular case diagnosis of TTP was established on a clinical basis, however, its confirmation by almost zero ADAMTS-13 activity arrived post-mortem, while HIV-infection was discovered post-mortem only and confirmed by immune blotting.

вирус иммунодефицита человека, ВИЧ-инфекция, тромботическая микроангиопатия, тромботическая тромбоцитопеническая пурпура, human immunodeficiency virus (HIV), HIV-infection, thrombotic microangiopathy (TMA), thrombotic thrombocytopenic purpura (TTP)

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