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Objectives: To review a single center outcome of patients with Langerhans Cell Histiocytosis diagnosed at a referral tertiary hospital from Turkey.
Methods: The files between 1989 and 2014 of 80 patients with LCH were retrospectively analyzed.
Results: During the 25 year period, 80 patients were diagnosed with LCH. The median age at diagnosis was 53 months (2-180 months) and median follow-up time of patients was 10 years and 9 months (24 months-25 years). Bone was the most frequently affected organ (n:60, 75%). Initially, 43 patients (54%) had single system (SS) disease, 20 patients (25%) had multisystem (MS) disease without risk organ involvement (MS-RO-), and 17 patients (21%) had multisystem disease with risk organ involvement (MS-RO+). The overall survival (OS) rate was 91% and event-free survival (EFS) rate was 67% at 10 years. The overall survival rate was lower in patients with RO involvement (p=<0.001), lack of response to systemic chemotherapy on 12th week (p=<0.001), younger age (<2 years) at presentation (p=<0.02), and skin involvement (<0.001).
Discussion: Besides the known risk factors like younger age (<2 years) at presentation, multi system risk organ involvement, and lack of response to initial chemotherapy, skin involvement was also associated with unfavorable outcome in our cohort. Evaluation of large cohorts of patients is needed to investigate this further.