The Turkish Journal of Pediatrics (Aug 2016)

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

  • Dilek İnce,
  • Selcen Kundak,
  • Yöntem Yaman,
  • Serra Kamer,
  • Yeşim Oymak,
  • Ragıp Ortaç,
  • Ayşe Erbay,
  • Gülcihan Özek,
  • Bengü Demirağ,
  • Canan Vergin

DOI
https://doi.org/10.24953/turkjped.2016.04.001
Journal volume & issue
Vol. 58, no. 4

Abstract

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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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