Langerhans cell histiocytosis: Single center experience of 25 years


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Tuysuz G., Yildiz I., Ozdemir N., Adaletli I. , Kurugoglu S. , Apak H. , ...Daha Fazla

Mediterranean Journal of Hematology and Infectious Diseases, cilt.11, sa.1, 2019 (SCI Expanded İndekslerine Giren Dergi) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 11 Konu: 1
  • Basım Tarihi: 2019
  • Doi Numarası: 10.4084/mjhid.2019.035
  • Dergi Adı: Mediterranean Journal of Hematology and Infectious Diseases

Özet

© 2019 Universita Cattolica del Sacro Cuore. All rights reserved.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.