Pediatric hemophagocytic lymphohistiocytosis in a tropical country: Results of a multicenter study in Thailand. Issue 2 (17th June 2022)

• Record Type: Journal Article
• Authors: Rungrojjananon, Nattapol; Pakakasama, Samart; Winaichatsak, Angkana; Siriwanawong, Rapee; Rujkijyanont, Piya; Traivaree, Chanchai; Photia, Apichat; Monsereenusorn, Chalinee
• Is Part Of: Asia-Pacific journal of clinical oncology. Volume 19:Issue 2(2023)
• Page Start: e128
• Page End: e137
• Publication Date: 2022-06-17
• Subjects: hemophagocytic lymphohistiocytosis -- outcome -- platelet count -- prognostic factors -- Thailand; Oncology -- Pacific Area -- Periodicals; Cancer -- Treatment -- Pacific Area -- Periodicals; Cancer -- Pacific Area -- Periodicals; Cancer -- Treatment -- Periodicals; 616.9940095
• DOI: 10.1111/ajco.13805
• Languages: English
• ISSNs: 1743-7555

Abstract

Abstract: Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life‐threatening condition caused by genetic mutation or various triggers disturbing the immune system. Methods: A multicenter retrospective study of pediatric patients with HLH receiving a diagnosis between January 2005 and December 2019 from three pediatric oncology centers was conducted to explore the clinical characteristics and determine prognostic factors associated with outcomes among Thai children. Results: In all, 78 patients with HLH with a median age at diagnosis of 3.17 (range, .08–17.83) years were enrolled. The male to female ratio was 1.2:1. The most common type of HLH was infection‐associated hemophagocytic syndrome (IAHS) ( n = 59, 75%) of which Epstein‐Barr virus was the most common pathogen. Thrombocytopenia, hyperbilirubinemia, and treatment response at weeks 2 and 8 after initiating treatment were associated with mortality. Platelet count <50, 000 cells/mm 3 was the only independent prognostic factor to define survival outcome ( p‐ value .035). Two‐year overall survival rate was 71.3% (95% confidence interval, 59.2%–80.3%). Survival rates between IAHS, malignant associated HLH, macrophage activation syndrome, and unspecific HLH did not significantly differ ( p ‐value .571). Conclusion: IAHS was the most common cause among pediatric HLH in Thailand. The outcomes of Thai children with HLH were comparable to those of developed countries. Platelet count <50, 000 cells/mm 3 was the only independent prognostic factor to define survival outcome. Abstract : The predictors and outcomes of Thai pediatric HLH were demonstrated. The outcomes were comparable to those of developed countries.