Etiologies and long-term outcome of pediatric hemophagocytic lymphohistiocytosis and macrophage activation syndrome in Taiwan: a single-center retrospective study.

ABSTRACT: Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are life-threatening hyperinflammatory conditions. Primary HLH is caused by genetic mutations associated with defective cytotoxicity, while secondary HLH is triggered by various factors, including infection-associated HLH (IAHS), rheumatic diseases-associated HLH (MAS), or malignancy-associated HLH (M-HLH). We retrospectively reviewed the medical records of patients younger than 20 years of age with physician-diagnosed HLH or MAS between January 2005 and July 2022 in a large medical center in Taiwan. Seven patients were prospectively enrolled since Jan 2019. Clinical and laboratory features, treatments rendered, and outcomes of patients with HLH/MAS were analyzed. Fifty-two patients with HLH/MAS were included

SUBMITTER: Wang CY 

PROVIDER: S-EPMC12284797 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature