Department of Molecular Medicine and Haematology, Faculty of Health Sciences, University of Witwatersrand and National Health Laboratory Service, Johannesburg, South Africa.
Department of Haematology, University of Stellenbosch and National Health Laboratory Service, Cape Town, South Africa.
HIV Med. 2022 Nov;23(10):1033-1040. doi: 10.1111/hiv.13305. Epub 2022 Apr 3.
Thrombotic thrombocytopenic purpura (TTP), a serious thrombotic microangiopathy (TMA), is prevalent in the South African HIV-infected population. The exact pathogenesis of HIV-associated TTP (HIV-TTP) is however still unclear with diagnostic and therapeutic inconsistancies.
A systematic review of the published literature regarding HIV-TTP was performed.
HIV-TTP is still associated with significant morbidity and mortality in Africa despite the availability of anti-retroviral therpy (ART). Diagnosis of HIV-TTP requires the presence of a micro-angiopathic haemolytic anaemia with significant red blood cell schistocytes and thrombocytopenia in the absence of another TMA but background activation of the coagulation system and inflammation in HIV infected people can result in diagnostic anbiguity. Plasma therapy in the form of infusion or exchange is successful but expensive, associated with side-effects and not widely available. Adjuvant immunosuppression therapy may of benefit in patients with HIV-TTP and ART must always be optimised. Endothelial dysfunction caused by chronic inflammation and complement activation most likely contributes to the development of HIV-TTP.
The role of adjuvant immunomodulating therpy, the therapeutic targets and pathogenic contribution from endothelial dysfunction in HIV-TTP requires further investigation.
血栓性血小板减少性紫癜（TTP）是一种严重的血栓性微血管病（TMA），在南非 HIV 感染人群中较为普遍。然而，HIV 相关性 TTP（HIV-TTP）的确切发病机制仍不清楚，存在诊断和治疗上的不一致。
对有关 HIV-TTP 的已发表文献进行系统回顾。
尽管有抗逆转录病毒治疗（ART），但 HIV-TTP 在非洲仍与显著的发病率和死亡率相关。HIV-TTP 的诊断需要存在微血管性溶血性贫血，伴有大量红细胞碎片和血小板减少，而无其他 TMA，但 HIV 感染者的凝血系统和炎症背景激活可能导致诊断上的模糊性。以输注或交换形式进行的血浆治疗是成功的，但昂贵，且伴有副作用，并且并不广泛可用。辅助免疫抑制治疗可能对 HIV-TTP 患者有益，而 ART 必须始终得到优化。慢性炎症和补体激活引起的内皮功能障碍很可能导致 HIV-TTP 的发生。
辅助免疫调节治疗的作用、治疗靶点以及 HIV-TTP 中内皮功能障碍的发病机制贡献需要进一步研究。
