HLH as an additional warning sign of inborn errors of immunity beyond familial-HLH in children: a systematic review.

ABSTRACT:

Background

Hemophagocytic Lymphohistiocytosis (HLH) is a rare and life-threatening condition characterized by a severe impairment of the immune homeostasis. While Familial-HLH (FHL) is a known cause, the involvement of other Inborn Errors of Immunity (IEI) in pediatric-HLH remains understudied.

Objective

This systematic review aimed to assess the clinical features, triggers, laboratory data, treatment, and outcomes of pediatric HLH patients with IEI other than FHL (IEInotFHL), emphasizing the importance of accurate identification and management.

Methods

A systematic search for studies meeting inclusion criteria was conducted in PubMed, EMBASE, MEDLINE, and Cochrane Central. Quality assessment was performed through JBI criteria.

Results

A comprehensive search yi

SUBMITTER: Ricci S 

PROVIDER: S-EPMC10896843 | biostudies-literature | 2024

REPOSITORIES: biostudies-literature