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

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...

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Published in:Asia-Pacific journal of clinical oncology 2023-04, Vol.19 (2), p.e128-e137
Main Authors: Rungrojjananon, Nattapol, Pakakasama, Samart, Winaichatsak, Angkana, Siriwanawong, Rapee, Rujkijyanont, Piya, Traivaree, Chanchai, Photia, Apichat, Monsereenusorn, Chalinee
Format: Article
Language:English
Subjects:
Adolescent
Cell activation
Child
Diagnosis
Epstein-Barr virus
Epstein-Barr Virus Infections - complications
Epstein-Barr Virus Infections - drug therapy
Female
hemophagocytic lymphohistiocytosis
Hemophagocytic syndrome
Herpesvirus 4, Human
Histiocytosis
Humans
Hyperbilirubinemia
Immune system
Lymphocytosis
Lymphohistiocytosis, Hemophagocytic - diagnosis
Lymphohistiocytosis, Hemophagocytic - drug therapy
Lymphohistiocytosis, Hemophagocytic - etiology
Macrophages
Male
Medical prognosis
outcome
Patients
Pediatrics
platelet count
Platelets
prognostic factors
Retrospective Studies
Thailand
Thailand - epidemiology
Thrombocytopenia
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Summary: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 
ISSN:1743-7555
1743-7563
DOI:10.1111/ajco.13805