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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...
Autores principales: | , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Università Cattolica del Sacro Cuore
2019
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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 |
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author | Tuysuz, G. Yildiz, I. Ozdemir, N. Adaletli, İ. Kurugoglu, S. Apak, H. Dervisoglu, S. Bozkurt, S. Celkan, T. |
author_facet | Tuysuz, G. Yildiz, I. Ozdemir, N. Adaletli, İ. Kurugoglu, S. Apak, H. Dervisoglu, S. Bozkurt, S. Celkan, T. |
author_sort | Tuysuz, G. |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-6548207 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Università Cattolica del Sacro Cuore |
record_format | MEDLINE/PubMed |
spelling | pubmed-65482072019-06-14 Langerhans Cell Histiocytosis: Single Center Experience of 25 Years Tuysuz, G. Yildiz, I. Ozdemir, N. Adaletli, İ. Kurugoglu, S. Apak, H. Dervisoglu, S. Bozkurt, S. Celkan, T. Mediterr J Hematol Infect Dis Original Article 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. Università Cattolica del Sacro Cuore 2019-05-01 /pmc/articles/PMC6548207/ /pubmed/31205639 http://dx.doi.org/10.4084/MJHID.2019.035 Text en This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by-nc/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Original Article Tuysuz, G. Yildiz, I. Ozdemir, N. Adaletli, İ. Kurugoglu, S. Apak, H. Dervisoglu, S. Bozkurt, S. Celkan, T. Langerhans Cell Histiocytosis: Single Center Experience of 25 Years |
title | Langerhans Cell Histiocytosis: Single Center Experience of 25 Years |
title_full | Langerhans Cell Histiocytosis: Single Center Experience of 25 Years |
title_fullStr | Langerhans Cell Histiocytosis: Single Center Experience of 25 Years |
title_full_unstemmed | Langerhans Cell Histiocytosis: Single Center Experience of 25 Years |
title_short | Langerhans Cell Histiocytosis: Single Center Experience of 25 Years |
title_sort | langerhans cell histiocytosis: single center experience of 25 years |
topic | Original Article |
url | 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 |
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