Open AccessClinical characteristics and disease course in children with haemophagocytic lymphohistiocytosis treated at the University children’s hospital in Belgrade
Author(s) -
Lidija Krivokapic-Dokmanovic,
Nada Krstovski,
Srdja Janković,
Jelena Lazić,
Nedeljko Radlović,
Dragana Janić
Publication year - 2012
Publication title -
srpski arhiv za celokupno lekarstvo
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.135
H-Index - 17
eISSN - 2406-0895
pISSN - 0370-8179
DOI - 10.2298/sarh1204191k
Subject(s) - medicine , macrophage activation syndrome , pediatrics , perforin , hemophagocytic lymphohistiocytosis , jaundice , disease , juvenile rheumatoid arthritis , immunology , cd8 , antigen
Haemophagocytic lymphohistiocytosis (HLH) is a disorder characterised by long-standing fever, splenomegaly and bicytopoenia or pancytopoenia. Lymphadenopathy, jaundice and neurological symptoms mayalsooccur. HLH may ensue in various forms of innate or acquired immunodeficiency with impaired cytotoxic lymphocyte function resulting in excessive macrophage activation.