Langerans Cell Histiocytosis: Single Center Experience of 25 Years

Abstract

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.

Langerhans cell histiocytosis in children: A retrospective case series of 126 cases

“…In the current study, the age at presentation was less than 5 years and the median age of diagnosis was 3 years, in concordance with the pediatric literature 1,2,11 . While a few studies show male predominance, 2,12 our study and others 13,14 documented more cases of female children.…”

“…Studies have shown the incidence of LCH in children to be 2 to 9 cases per million per year with a male preponderance. LCH has a peak incidence at 1‐4 years of age, and children under 1 year are affected more severely 1‐3 . Around 2% cases present in the neonatal age group.…”

“…are affected more severely. [1][2][3] Around 2% cases present in the neonatal age group. Familial cases have been reported but without any specific genetic association.…”

Langerhans cell histiocytosis in children under 12 months of age: The spectrum of imaging and clinical findings: Experience in an Irish tertiary referral centre

“…A retrospective review of a database of patients with LCH over 25 years at a tertiary referral centre in Turkey demonstrated that the most affected organs after bone were skin, soft tissue and liver [6]⋅ This is in line with other reports in the literature.…”

Langerhans cell histiocytosis: A case report with oral manifestations and the role of pediatric dentists in the diagnosis

“…Close follow‐up is mandatory to rule out the recurrence of the disease, which depends on the degree of tissue involvement and method of treatment. The ten‐year survival rate for a single bone involvement has been reported to be 100% …”