Monsereenusorn C.Suwannaying K.Buaboonnam J.Sathitsamitphong L.Techavichit P.Pakakasama S.Chainansamit S.o.Anurathapan U.Komvilaisak P.Traivaree C.Sanpakit K.Charoenkwan P.Seksarn P.Mahidol University2024-06-102024-06-102024-05-01Asian Pacific journal of cancer prevention : APJCP Vol.25 No.5 (2024) , 1831-1839https://repository.li.mahidol.ac.th/handle/20.500.14594/98681BACKGROUND: Disease reactivation/refractory remains a major challenge in managing Langerhans cell histiocytosis (LCH). Outcomes and late sequelae should be explored. METHODS: A multi-institutional retrospective study was conducted to describe clinical characteristics, predictive factors, outcomes and late sequelae of pediatric reactivation/refractory LCH in Thailand. RESULTS: In all, 47 patients were studied, 25 (53.2%) patients had disease reactivation and 22 (46.8%) patients had refractory LCH. The median reactivation and refractory time were 1.59 and 0.33 years from diagnosis, respectively (p <0.001). The most common site of reactivation/refractory was the bone (n = 26, 55%), and 20 (42.6%) patients developed late sequelae. The 5-year overall survival (OS) was 76.1%. Patients with reactivation and refractory LCH performed similarly in 5-year OS (88% vs. 63%, p = 0.055). Prognostic factors associated with mortality were liver, spleen, hematopoietic system and lung reactivation (p <0.05). Lung reactivation was the only independent risk factor associated with the survival outcome (p = 0.002). CONCLUSIONS: The outcomes of pediatric patients between reactivation and refractory LCH in Thailand were similarly desirable and mortality was minimal although late sequelae may evolve. Pulmonary reactivation/refractory was an independent risk factor associated with survival.Biochemistry, Genetics and Molecular BiologyMedicineNationwide Study of Factors Impacting Survival Outcome and Consequences in Children with Reactivation/Refractory Langerhans Cell HistiocytosisArticleSCOPUS10.31557/APJCP.2024.25.5.18312-s2.0-851948595612476762X38809656