Langerhans Cell Histiocytosis in Children: A Single Center Experience from Turkey

The aim of the study is to present the experience of children diagnosed with Langerhans cell histiocytosis (LCH) at our center. Medical records of children with LCH were screened in terms of gender, age at diagnosis, clinical findings, risk groups, treatment modalities and outcome. The study included 9 patients with a median age of 22 months. The most common clinical finding was bone lesions (%88,8), followed by hepatosplenomegaly (%44.4), lymphadenopathy (%44.4), seborrheic dermatitis (33,3%) and jaundice (33.3%). Of the 9 patients, 3 (33.3%) had single system disease [1 (11.1%) had unifocal while 2 (22.2%) had multifocal disease], 6 (66.6%) had multisystem disease [2 (22.2%) had risk organ negative and 4 (44.4%) had risk organ positive]. At the end of the 6th week of therapy, 5 patients (55.5%) had all the dead children had better response, 1 (11.1%) had an intermediate response while 3 (33.3%) had worse response. In terms of outcome, 5 (55%) patients were in complete remission, 3 (33%) patients were dead, and 1 patient was lost to follow up. All dead children had multisystem disease with risk organ involvement and were worse responders at the 6th week of therapy. In children with LCH, the most important factors determining prognosis were risk organ involvement, multisystem disease and response at 6th week of treatment. New treatment modalities such as new drugs or stem cell transplantation are required for children with risk organ positive multisystem disease, as the prognosis remains poor.