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

Southern African journal of HIV medicine Pub Date : 2023-10-27 eCollection Date: 2023-01-01 DOI:10.4102/sajhivmed.v24i1.1504
Yusuf Moola, Zaheera Cassimjee, Chandni Dayal, Sheetal Chiba, Adekunle Ajayi, Malcolm Davies
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Abstract

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.

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HIV时代的血栓性血小板减少性紫癜:一个单一中心的经验。
背景:血栓性血小板减少性紫癜(TTP)是一种罕见的高死亡率疾病。HIV是TTP的重要原因。目的:我们通过HIV状态评估血浆置换(PEX)的表现和反应。方法:对2010年1月1日至2019年12月31日期间接受TTP PEX治疗的所有患者进行单中心回顾性审查。根据情况,使用Mann-Whitney U和Kruskal-Wallis方差检验分析,比较HIV相关TTP和其他病因之间的人口学和表现参数。病因和呈现参数对PEX持续时间的影响使用Cox比例风险建模;采用逐步多元回归分析这些变量对幸存者死亡率和残余肾功能不全的影响。结果:在83例患者中,不受控制的HIV感染是TTP的最常见原因(81.9%)。在HIV相关TTP中,血小板减少更严重,神经系统缺陷更常见;但本组肾功能不全较轻。病因不影响死亡率。病因类别和表现参数不能预测PEX的持续时间。残余肾功能不全在HIV相关TTP的幸存者中发生率较低。结论:HIV是TTP的一个重要原因。血液学和神经系统受累在HIV相关TTP中更为严重。即使在晚期HIV感染中,PEX也可以达到可接受的存活率;肾亮片在这一组中不太常见。
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