Pediatric hemophagocytic lymphohistiocytosis in a tropical country: Results of a multicenter study in Thailand

噬血细胞性淋巴组织细胞增多症 医学 置信区间 巨噬细胞活化综合征 儿科 内科学 噬血作用 多中心研究 免疫学 疾病 随机对照试验 全血细胞减少症 骨髓
作者
Nattapol Rungrojjananon,Samart Pakakasama,Angkana Winaichatsak,Rapee Siriwanawong,Piya Rujkijyanont,Chanchai Traivaree,Apichat Photia,Chalinee Monsereenusorn
出处
期刊:Asia-pacific Journal of Clinical Oncology [Wiley]
标识
DOI:10.1111/ajco.13805
摘要

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition caused by genetic mutation or various triggers disturbing the immune system.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.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/mm3 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).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/mm3 was the only independent prognostic factor to define survival outcome.
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