1. Nationwide Study of Factors Impacting Survival Outcome and Consequences in Children with Reactivation/Refractory Langerhans Cell Histiocytosis.
- Author
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Monsereenusorn C, Suwannaying K, Buaboonnam J, Sathitsamitphong L, Techavichit P, Pakakasama S, Chainansamit SO, Anurathapan U, Komvilaisak P, Traivaree C, Sanpakit K, Charoenkwan P, and Seksarn P
- Subjects
- Humans, Male, Female, Retrospective Studies, Child, Prognosis, Child, Preschool, Thailand epidemiology, Survival Rate, Infant, Follow-Up Studies, Adolescent, Risk Factors, Histiocytosis, Langerhans-Cell mortality, Histiocytosis, Langerhans-Cell pathology
- Abstract
Background: 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.
- Published
- 2024
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