Migaud Pascal, Dalla Pria Alessia, Hosmann Kai, Kelleher Peter, Fulgenzi Claudia Anna Maria, Stocker Hartmut, Bower Mark
Department of Infectious Diseases, St-Joseph Hospital, Berlin-Tempelhof.
Charité Universitätsmedizin Berlin, Berlin, Germany.
AIDS. 2025 Apr 1;39(5):519-525. doi: 10.1097/QAD.0000000000004094. Epub 2024 Dec 16.
The clinical and laboratory characteristics of HHV-8-associated multicentric Castleman disease (MCD) in people with HIV (PWH) overlap with those of haemophagocytic lymphohistiocytosis (HLH) disease and indeed the two diagnoses may co-exist. A risk-stratified treatment approach to MCD based on Rituximab immunotherapy for mild cases and chemo-immunotherapy for severe cases has been shown to yield excellent outcomes in PWH. In contrast, HLH disease, previously known as secondary HLH, has a dismal prognosis even when promptly treated according to guidelines.
A retrospective multicentre cohort study.
Retrospective analysis of prospectively collected clinical and pathological data on patients with biopsy-proven HIV-associated MCD at the National Centre for HIV Malignancy at Chelsea and Westminster Hospital, London between 2008 and 2024 and at the Department of Infectious Diseases at St. Joseph Hospital Berlin-Tempelhof, Germany between 2020 and 2024.
In our UK-German cohort, including 113 PWH with MCD, we confirmed that HLH disease secondary to MCD is common (30%), and we demonstrated that HLH disease in this context does not adversely influence survival or risk of MCD relapse.
We suggest that a high HScore in MCD should not lead to a change in the treatment in this specific setting.
人类免疫缺陷病毒（HIV）感染者（PWH）中与HHV-8相关的多中心Castleman病（MCD）的临床和实验室特征与噬血细胞性淋巴组织细胞增生症（HLH）重叠，实际上这两种诊断可能同时存在。基于利妥昔单抗免疫疗法治疗轻症MCD以及化疗免疫疗法治疗重症MCD的风险分层治疗方法已被证明在PWH中能产生优异的治疗效果。相比之下，HLH病（以前称为继发性HLH），即使根据指南及时治疗，预后也很差。
一项回顾性多中心队列研究。
对2008年至2024年期间伦敦切尔西和威斯敏斯特医院国家HIV恶性肿瘤中心以及2020年至2024年期间德国柏林-滕珀尔霍夫圣约瑟夫医院传染病科前瞻性收集的经活检证实的HIV相关MCD患者的临床和病理数据进行回顾性分析。
在我们的英德队列中，包括113例患有MCD的PWH，我们证实MCD继发的HLH病很常见（30%），并且我们证明在这种情况下HLH病不会对生存或MCD复发风险产生不利影响。
我们建议在这种特定情况下，MCD中高HScore不应导致治疗改变。
