Hemophagocytic syndrome: Clinical characterization and follow-up of a Chilean pediatric cohort

• Published in: Revista chilena de infectología Vol. 38; no. 3; pp. 423 - 431
• Main Authors: Astudillo P, Patricio, Parejas T, Carla, Wietstruck P, María Angélica, Morales M, Pamela, Abarca V, Katia
• Format: Journal Article
• Language: Spanish
• Published: 01.06.2021
• ISSN: 0717-6341
• DOI: 10.4067/S0716-10182021000300423

BACKGROUNDHemophagocytic lymphohistiocytosis (HLH) is a severe syndrome, potentially lethal, with a pathological activation of the immune system and an extreme hyperinflammatory response. The etiology is classified in primary HLH (familiar or genetic) and secondary (infectious, oncological, and rheumatological diseases). AIMTo analyze clinical and laboratory characteristics, treatment, and follow-up rates in pediatric patients with HLH. METHODSA pediatric cohort of patients with HLH diagnosis attending in a tertiary hospital between January 2000 to February 2019 was analysed. RESULTS23 hospitalized patients were recruited with a median of 36 months of age. The most frequent clinical and laboratory findings were fever, cytopenias, and hyperferritinemia. The most frequent aetiologies were infectious (Epstein Barr virus and citomegalovirus) and rheumatological diseases. The global mortality was 35%, there was no significant difference between etiologies. DISCUSSIONConsidering the high mortality of HLH it is very important to have a high grade of suspicion that allows treating at an early stage. It would be important to determine clinical and laboratory predictors in multicentric studies.