Hemophagocytic lymphohistiocytosis in HIV-associated HHV8-positive multicentric castleman disease.

IF 3.4 2区 医学 Q3 IMMUNOLOGY AIDS Pub Date : 2024-12-17 DOI:10.1097/QAD.0000000000004094
Pascal Migaud, Alessia Dalla Pria, Kai Hosmann, Peter Kellerher, Claudia Anna Maria Fulgenzi, Hartmut Stocker, Mark Bower
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Abstract

Objective: The clinical and laboratory characteristics of HHV8-associated Multicentric Castleman Disease (MCD) in people living with HIV (PLWH) overlap with those of Hemophagocytic 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 PLWH. In contrast, HLH disease, previously known as secondary HLH, has a dismal prognosis even when promptly treated according to guidelines.

Design: A retrospective multicentre cohort study.

Methods: 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.

Results: In our UK-German cohort including 113 PWLH 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.

Conclusion: We suggest that a high HScore in MCD should not lead to a change in the treatment in this specific setting.

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hiv相关hhv8阳性多中心castleman病的噬血细胞淋巴组织细胞增多症
目的:HIV感染者(PLWH) hhv8相关多中心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多症(HLH)疾病的临床和实验室特征重叠,实际上这两种诊断可能共存。一种基于利妥昔单抗免疫治疗的轻度MCD风险分层治疗方法和基于化疗免疫治疗的重症PLWH已显示出良好的疗效。相比之下,HLH疾病,以前被称为继发性HLH,即使根据指南及时治疗,预后也很差。设计:回顾性多中心队列研究。方法:回顾性分析2008年至2024年在伦敦切尔西和威斯敏斯特医院国家HIV恶性肿瘤中心和2020年至2024年在德国柏林- tempelhof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。结果:在我们的英国-德国队列中,包括113名患有MCD的PWLH,我们证实了继发于MCD的HLH疾病是常见的(30%),我们证明了在这种情况下HLH疾病不会对生存或MCD复发风险产生不利影响。结论:我们认为MCD的高HScore不应该导致在这种特殊情况下治疗的改变。
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来源期刊
AIDS
AIDS 医学-病毒学
CiteScore
5.90
自引率
5.30%
发文量
478
审稿时长
3 months
期刊介绍: ​​​​​​​​​​​​​​​​​Publishing the very latest ground breaking research on HIV and AIDS. Read by all the top clinicians and researchers, AIDS has the highest impact of all AIDS-related journals. With 18 issues per year, AIDS guarantees the authoritative presentation of significant advances. The Editors, themselves noted international experts who know the demands of your work, are committed to making AIDS the most distinguished and innovative journal in the field. Submitted articles undergo a preliminary review by the editor. Some articles may be returned to authors without further consideration. Those being considered for publication will undergo further assessment and peer-review by the editors and those invited to do so from a reviewer pool.
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