Hemophagocytic lymphohistiocytosis in people living with HIV-a single centre experience.

IF 5.4 2区医学 Q1 INFECTIOUS DISEASES

Infection Pub Date : 2025-06-03 DOI:10.1007/s15010-025-02573-1

Pascal Migaud, Daniela Drauz, Alessia Dalla Pria, Kai Hosmann, Markus Müller, Leyli Ghaeni, Hartmut Stocker

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引用次数: 0

Abstract

Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome that clinically resembles sepsis thus obscuring the underlying condition and delaying its diagnosis and therapy. Among the most common triggers are lymphomas and infectious diseases. Lymphoma-associated HLH appears to be more common in People living with HIV (PLWH).

Methods: Retrospective cohort study comprising all adult HIV-infected patients with HLH treated at St. Joseph Hospital Berlin-Tempelhof, Germany, defined by HLH 2004-criteria and the HScore, between April 2020 and November 2024.

Results: 22 patients were included with at least 5/8 positive HLH criteria and a median HScore of 222 points. Median age was 44 [29-66] years. The median CD4-count at HLH-diagnosis was 100/µL [14-936]. In 8 (36%) patients the HIV-viral load was undetectable. HLH led to the diagnosis of HIV in 6 (27%) patients. In 20/22 patients an LPD was the HLH trigger. Hodgkin's lymphoma, HHV8-positive multicentric Castleman disease and HHV8-positive primary effusion lymphoma accounted for 8 (36%), 5 (23%) and 3 (14%) cases respectively. Kaposi sarcoma inflammatory cytokine syndrome (KICS) HHV8-positive plasmablastic lymphoma, HHV8-positive diffuse large B-cell lymphoma, DLBCL and invasive Aspergillosis were each found in 1 (4%) patient. All patients with Hodgkin's lymphoma had bone marrow involvement. In 1 patient simultaneous malaria and multiple myeloma were diagnosed. 11/22 (50%) patients had HHV8-associated conditions. 5 (23%) patients died within 30 days of the HLH-diagnosis.

Conclusion: Lymphomas and HHV8-associated diseases are common triggers of HLH in PLWH and are linked to a high mortality rate.

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艾滋病毒感染者的噬血细胞性淋巴组织细胞增多症——单中心经验。

背景：噬血细胞性淋巴组织细胞增多症（HLH）是一种危及生命的高炎症综合征，临床上类似于脓毒症，因此掩盖了潜在的疾病，延误了其诊断和治疗。最常见的诱因是淋巴瘤和传染病。淋巴瘤相关的HLH似乎在HIV感染者（PLWH）中更为常见。方法：回顾性队列研究纳入了2020年4月至2024年11月期间在德国柏林- tempelhof圣约瑟夫医院接受HLH治疗的所有成年hiv感染的HLH患者，根据HLH 2004-标准和HScore进行定义。结果：22例患者至少有5/8阳性HLH标准，中位HScore为222分。中位年龄44岁[29-66]岁。hlh诊断时cd4中位数为100/µL[14-936]。8例（36%）患者无法检测到hiv病毒载量。6例（27%）患者被HLH诊断为HIV。在20/22的患者中，LPD是HLH的触发因素。霍奇金淋巴瘤8例（36%），hhv8阳性多中心Castleman病5例（23%），原发性积液性淋巴瘤3例（14%）。卡波西肉瘤炎性细胞因子综合征（KICS） hhv8阳性浆母细胞淋巴瘤、hhv8阳性弥漫性大b细胞淋巴瘤、DLBCL和侵袭性曲霉病各1例（4%）。所有霍奇金淋巴瘤患者均有骨髓受累。1例患者同时诊断为疟疾和多发性骨髓瘤。11/22（50%）患者有hhv8相关疾病。5例（23%）患者在确诊后30天内死亡。结论：淋巴瘤和hhv8相关疾病是PLWH患者HLH的常见诱因，并与高死亡率相关。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Infection 医学-传染病学

CiteScore

12.50

自引率

1.30%

发文量

224

审稿时长

6-12 weeks

期刊介绍： Infection is a journal dedicated to serving as a global forum for the presentation and discussion of clinically relevant information on infectious diseases. Its primary goal is to engage readers and contributors from various regions around the world in the exchange of knowledge about the etiology, pathogenesis, diagnosis, and treatment of infectious diseases, both in outpatient and inpatient settings. The journal covers a wide range of topics, including: Etiology: The study of the causes of infectious diseases. Pathogenesis: The process by which an infectious agent causes disease. Diagnosis: The methods and techniques used to identify infectious diseases. Treatment: The medical interventions and strategies employed to treat infectious diseases. Public Health: Issues of local, regional, or international significance related to infectious diseases, including prevention, control, and management strategies. Hospital Epidemiology: The study of the spread of infectious diseases within healthcare settings and the measures to prevent nosocomial infections. In addition to these, Infection also includes a specialized "Images" section, which focuses on high-quality visual content, such as images, photographs, and microscopic slides, accompanied by brief abstracts. This section is designed to highlight the clinical and diagnostic value of visual aids in the field of infectious diseases, as many conditions present with characteristic clinical signs that can be diagnosed through inspection, and imaging and microscopy are crucial for accurate diagnosis. The journal's comprehensive approach ensures that it remains a valuable resource for healthcare professionals and researchers in the field of infectious diseases.