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Langerhans Cell Histiocytosis: Single Center Experience of 25 Years

OBJECTIVES: To review a single center outcome of patients with Langerhans Cell Histiocytosis diagnosed at a tertiary referral hospital from Turkey. Methods: The files between 1989 and 2015 of 80 patients with LCH were retrospectively analyzed. RESULTS: During the 25 years, 80 patients were diagnosed...

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Detalles Bibliográficos
Autores principales: Tuysuz, G., Yildiz, I., Ozdemir, N., Adaletli, İ., Kurugoglu, S., Apak, H., Dervisoglu, S., Bozkurt, S., Celkan, T.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Università Cattolica del Sacro Cuore 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6548207/
https://www.ncbi.nlm.nih.gov/pubmed/31205639
http://dx.doi.org/10.4084/MJHID.2019.035
Descripción
Sumario:OBJECTIVES: To review a single center outcome of patients with Langerhans Cell Histiocytosis diagnosed at a tertiary referral hospital from Turkey. Methods: The files between 1989 and 2015 of 80 patients with LCH were retrospectively analyzed. RESULTS: During the 25 years, 80 patients were diagnosed with LCH. The median age at diagnosis was 53 months (2–180 months) and the 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 a 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. 10-year OS rate was lower for patients with MS-RO(+) (65%) when compared to those with, MS-RO(−), and SS (100%, 97%, p value=<0.001). The overall survival rate was also lower in patients with lack of response to systemic chemotherapy on 12th week (p=<0.001), younger age (<2 years) at presentation (p=<0.02), skin involvement (<0.001) and lack of bone lesions at presentation (<0.001). DISCUSSION: In the group with MS-RO(+), OS is significantly low compared to other groups. Further efforts are warranted to improve survival in MS-RO(+) patients.