Outcome of Childhood Langerhans Cell Histiocytosis: Experience from a Lower-Middle Income Country
DOI:
https://doi.org/10.36283/ziun-pjmd15-2/003Keywords:
Langerhans cell histiocytosis, Lower-middle income country , Pakistan , Pediatric oncology , Resource-limited setting , Risk organ , SurvivalAbstract
Background: Langerhans cell histiocytosis (LCH) is an uncommon myeloid neoplasm, presenting with a wide spectrum of clinical manifestations. Prognosis varies widely based on disease extent and risk organ (RO) involvement. This study aimed to assess treatment outcomes and prognostic indicators in pediatric LCH.
Methods: A retrospective study was conducted at the Pediatric Hematology/Oncology Department, Combined Military Hospital (CMH) Rawalpindi. Twenty-nine children aged 0–18 years, diagnosed and treated from June 2013 to June 2023, were enrolled through non-probability consecutive sampling. Data were extracted retrospectively from medical records of patients and analyzed using SPSS version 25.0. Kaplan-Meier survival curves were generated for event-free survival (EFS) and overall survival (OS), and compared using the log-rank test. Prognostic factors were evaluated through the Cox regression analysis. A p-value of <0.05 was considered statistically significant.
Results: Among the 29 patients, 22 (75.86%) had multisystem disease, with 12 (54.54%) showing RO positivity. An age of 18 months represented the median at diagnosis. Relapse occurred in 6 (20.68%) patients; all were successfully re-treated. Eleven (37.93%) patients expired, all with multisystem disease, and 9 (81.81%) had RO involvement. With a median follow-up of 80.10 months, the rates of 5-year EFS and OS stood at 32% and 60%, respectively. Patients with RO involvement had significantly lower EFS and OS rates (p=0.001 and p=0.00, respectively).
Conclusion: Multisystem and RO positive cases have poor short-term outcomes, but long-term prognosis improves with survival beyond the initial 2-3 years. RO involvement remains the most powerful predictor of mortality and morbidity in LCH
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