Serra KAMER, Selcen KUNDAK, Ragıp ORTAÇ, Yöntem YAMAN, Bengü DEMİRAĞ, Canan VERGİN, Yeşim OYMAK, Gülcihan ÖZEK, Dilek İNCE, Ayşe ERBAY

Pediatric langerhans cell histiocytosis: single center experience over a 17-year period

İnce D, Demirağ B, Özek G, Erbay A, Ortaç R, Oymak Y, Kamer S, Yaman Y, Kundak S, Vergin C. Pediatric langerhans cell histiocytosis: single center experience over a 17-year period. Turk J Pediatr 2016; 58: 349-355.This study aimed to analyze children with the diagnosis of Langerhans cell histiocytosis (LCH) who were diagnosed and treated between 1998-2015. Medical records were evaluated retrospectively for clinical and laboratory features, treatment details, and outcome. There were 20 patients, the median age of diagnosis was 37 months, M/F ratio: 1.5. Nine had single system (SS), 11 had multisystem (MS) LCH. Spontaneous regression occurred in three infants with skin limited LCH. Eight patients had risk organ involvement in MS-LCH group. The curettage alone was performed in only one case. Patients received LCH-II/ LCH-III based chemotherapy schema. Radiotherapy was performed to vertebral disease and residual craniofacial bone disease in four cases.The regression and relapse rates were 100% and 33% for SS-LCH. The regression and relapse rates were 73%, and 18% for MS-LCH. Two infants with MS-LCH died despite chemotherapy.Pulmonary and liver involvements affected outcome adversely in MS-LCH. Multidisciplinary treatment approaches are needed.

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