Lan Tingwei, Luo Qinhuan, Guo Xiaojuan, Jiang Xuan
Tsinghua University, Tsinghua Medicine, School of Medicine, Beijing, China.
Beijing Tsinghua Changgung Hospital, Department of Gastroenterology, Beijing, China.
Front Immunol. 2025 Aug 15;16:1575297. doi: 10.3389/fimmu.2025.1575297. eCollection 2025.
Hemophagocytic lymphohistiocytosis (HLH) is a rare but life-threatening hyperinflammatory syndrome. Patients with inflammatory bowel diseases (IBD) appear to be at increased risk of developing HLH, potentially due to immunosuppressive therapies. However, the epidemiology, clinical characteristics, management strategies, and outcomes of HLH in this population remain poorly understood.
We conducted a systematic review of the literature using PubMed, Web of Science, and Embase. A total of 97 secondary HLH (sHLH) cases and 18 HLH cases with genetic mutations were identified in IBD patients.
Among IBD patients, sHLH predominantly affected males with Crohn's disease, with a median age of 33.5 years and a median disease duration of 4 years. Most patients were on thiopurines for IBD management and were in clinical remission at sHLH onset. The most common triggers were infections (particularly CMV and EBV), followed by lymphoma. The overall survival rate for sHLH was 62.5%. Most patients successfully resumed IBD maintenance therapy within 5 months after the sHLH episode, with minimal complications and rare recurrence of IBD or HLH. Older age, lymphoma-induced HLH, and lack of biologic or thiopurine therapy were potential factors associated with mortality. Compared to sHLH, primary HLH patients were younger, more frequently male, predominantly had Crohn's disease, were less likely to be in remission despite biologic therapy, and had better outcomes with hematopoietic stem cell transplantation (HSCT).
This study provides a comprehensive characterization of HLH in IBD patients, offering valuable insights to guide future research aimed at improving clinical outcomes in this unique population.
osf.io identifier, 10.17605/OSF.IO/5GY3E.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见但危及生命的高炎症综合征。炎症性肠病（IBD）患者发生HLH的风险似乎增加，这可能与免疫抑制治疗有关。然而，该人群中HLH的流行病学、临床特征、管理策略和结局仍知之甚少。
我们使用PubMed、Web of Science和Embase对文献进行了系统综述。在IBD患者中总共鉴定出97例继发性HLH（sHLH）病例和18例具有基因突变的HLH病例。
在IBD患者中，sHLH主要影响患有克罗恩病的男性，中位年龄为33.5岁，中位病程为4年。大多数患者因IBD治疗而使用硫唑嘌呤，在sHLH发病时处于临床缓解期。最常见的诱因是感染（尤其是巨细胞病毒和EB病毒），其次是淋巴瘤。sHLH的总体生存率为62.5%。大多数患者在sHLH发作后5个月内成功恢复IBD维持治疗，并发症极少，IBD或HLH复发罕见。年龄较大、淋巴瘤诱发的HLH以及缺乏生物制剂或硫唑嘌呤治疗是与死亡率相关的潜在因素。与sHLH相比，原发性HLH患者更年轻，男性更常见，主要患有克罗恩病，尽管接受生物治疗但缓解的可能性较小，造血干细胞移植（HSCT）的结局更好。
本研究全面描述了IBD患者中的HLH，为指导未来旨在改善这一独特人群临床结局的研究提供了有价值的见解。
osf.io标识符，10.17605/OSF.IO/5GY3E。
