| Literature DB >> 28276205 |
Dilek İnce1, Bengü Demirağ2, Gülcihan Özek2, Ayşe Erbay3, Ragıp Ortaç4, Yeşim Oymak2, Serra Kamer5, Yöntem Yaman2, Selcen Kundak6, Canan Vergin2.
Abstract
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.Entities:
Keywords: Langerhans cell histiocytosis; children; treatment
Mesh:
Year: 2016 PMID: 28276205 DOI: 10.24953/turkjped.2016.04.001
Source DB: PubMed Journal: Turk J Pediatr ISSN: 0041-4301 Impact factor: 0.552