Hemophagocytic lymphohistiocytosis in patients with inflammatory bowel diseases: a systematic review.

ABSTRACT:

Introduction

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.

Methods

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.

Results

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

SUBMITTER: Lan T 

PROVIDER: S-EPMC12394226 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature