Clinical Outcomes of Langerhans Cell Histiocytosis: A Single-Center Experience

Abstract

Objective: To evaluate the clinical outcome of Langerhans cell histiocytosis (LCH) in countries with limited resources. Material and Methods: A retrospective chart review of patients <15 years old, diagnosed with LCH and treated at Siriraj Hospital; from January 1, 2002 until December 31, 2016, was performed. The patients’ demographic data, treatment protocol, and efficacy of treatment were collected and analyzed. Results: LCH was diagnosed in 38 patients, with a median age of 1.9 years old; of whom, 24 had multisystem disease (MS) and 14 had single system disease (SS). In the MS group, 16 patients had risk of organ (RO) involvement, with hepatic involvement being most common. The prevalence of central diabetes insipidus was 21.0%. Reactivation was observed in 11 patients (28.9%). RO involvement of the hematopoietic system (p-value=0.016), spleen (p-value=0.031), and MS (p-value=0.001) were significantly associated with a poor response to induction therapy. RO involvement of the hematopoietic system (p-value=0.016), liver (p-value=0.0), and spleen (p-value=0.005), as well as MS (p-value=0.013) were significantly associated with reactivation risk. The 5-year event-free survival rate of patients with and without RO involvement were 9.2% and 88.3%, respectively. The 5-year overall survival rate of patients with and without RO involvement were 82.5% and 96.4%, respectively. Conclusion: The prevalence of MS and RO involvement seemed high in this cohort; however, the outcomes were comparable to other Asian studies. Novel treatment for RO involvement may improve clinical outcomes.