Multicenter validation of secondary hemophagocytic lymphohistiocytosis diagnostic criteria.

ABSTRACT:

Background

Five fulfilled hemophagocytic lymphohistiocytosis (HLH)-2004 criteria, and the HScore are widely used and recommended by international expert consensus to diagnose secondary HLH. Both diagnostic scores have never been validated in heterogeneous patient cohorts of secondary HLH patients. We aimed to systematically optimize and validate diagnostic criteria of secondary HLH using a multicenter approach.

Methods

We developed optimized criteria in our cohort of critically ill patients as a first step. We next validated these new criteria together with the original and modified HLH-2004 criteria as well as the HScore using original data of 13 published cohorts, which were identified by a systematic literature search.

Results

The best performing HLH diagnostic cri

SUBMITTER: Lachmann G 

PROVIDER: S-EPMC11846073 | biostudies-literature | 2025 Mar

REPOSITORIES: biostudies-literature