Migaud Pascal, Drauz Daniela, Dalla Pria Alessia, Hosmann Kai, Müller Markus, Ghaeni Leyli, Stocker Hartmut
Department of Infectious Diseases, St. Joseph Hospital, Berlin-Tempelhof, Germany.
Charité Universitätsmedizin, Berlin, Germany.
Infection. 2025 Jun 3. doi: 10.1007/s15010-025-02573-1.
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).
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.
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.
Lymphomas and HHV8-associated diseases are common triggers of HLH in PLWH and are linked to a high mortality rate.
噬血细胞性淋巴组织细胞增生症（HLH）是一种危及生命的高炎症综合征，临床上类似于败血症，从而掩盖了潜在病情并延误其诊断和治疗。最常见的触发因素包括淋巴瘤和传染病。淋巴瘤相关的HLH在艾滋病毒感染者（PLWH）中似乎更为常见。
回顾性队列研究，纳入2020年4月至2024年11月期间在德国柏林-滕珀尔霍夫圣约瑟夫医院接受治疗的所有成年HIV感染且患有HLH的患者，根据HLH 2004标准和HScore进行定义。
纳入22例患者，至少有5/8项HLH标准呈阳性，HScore中位数为222分。中位年龄为44[29 - 66]岁。HLH诊断时的CD4细胞计数中位数为100/µL[14 - 936]。8例（36%）患者的HIV病毒载量检测不到。HLH导致6例（27%）患者被诊断出感染HIV。20/22例患者中，淋巴增殖性疾病（LPD）是HLH的触发因素。霍奇金淋巴瘤、HHV8阳性多中心Castleman病和HHV8阳性原发性渗出性淋巴瘤分别占8例（36%）、5例（23%）和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、弥漫性大B细胞淋巴瘤（DLBCL）和侵袭性曲霉病在各1例（4%）患者中被发现。所有霍奇金淋巴瘤患者均有骨髓受累。1例患者同时被诊断出患有疟疾和多发性骨髓瘤。11/22（50%）例患者患有HHV8相关疾病。5例（23%）患者在HLH诊断后30天内死亡。
淋巴瘤和HHV8相关疾病是PLWH中HLH的常见触发因素，且与高死亡率相关。
