Thrombotic thrombocytopaenic purpura in the era of HIV: A single-centre experience

BACKGROUND: Thrombotic thrombocytopaenia purpura (TTP) is a rare disorder which carries a high mortality. HIV is an important cause of TTP. OBJECTIVES: We assessed the presentation and response to plasma exchange (PEX) by HIV status. METHOD: A single-centre retrospective review of all patients receiving PEX for TTP between 01 January 2010 and 31 December 2019 was undertaken. Demographics and presenting parameters were compared between HIV-associated TTP and other aetiologies using Mann-Whitney U and Kruskal Wallis analysis of variance testing, as appropriate. The effect of aetiology and presenting parameters on PEX duration was modelled using Cox proportional hazards; effect of these variables on mortality and residual renal dysfunction in survivors was analysed using stepwise multivariate regression. RESULTS: Uncontrolled HIV infection was the commonest cause (81.9%) of TTP in the 83 patients identified. Thrombocytopaenia was more severe and neurological deficit more frequent in HIV-associated TTP; but renal dysfunction was milder in this group. Aetiology did not influence mortality risk. Aetiological category and presenting parameters did not predict PEX duration. Residual renal dysfunction was less frequent in survivors of HIV-associated TTP. CONCLUSION: HIV is an important cause of TTP in the local context. Haematological and neurological involvement are more severe in HIV-associated TTP. Acceptable survival rates are achievable with PEX even in advanced HIV infection; renal sequalae are less common in this group.