Yıl: 2023 Cilt: 5 Sayı: 1 Sayfa Aralığı: 59 - 64 Metin Dili: İngilizce DOI: 10.37990/medr.1159055 İndeks Tarihi: 21-09-2023

Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience

Öz:
Aim: Langerhans cell histiocytosis (LCH) is a proliferative disease caused by abnormal proliferation of histiocytes in the bone marrow dendritic cell structure. LCH is very rare in adults and its incidence is 1-2 cases per million. Therefore, there is still no clear management plan in adults. It was aimed to evaluate the very rare adult LCH patients. Materials and Methods: Ten LCH patients who were followed up at Erciyes University Hematology Department between January 2010 and January 2020 were included. Results: Eight (80%) of them were male and 2 (20%) were female. The median age of the patients was 34.5 ± 8.4 (23-52) years. Although the most common involvement at the time of diagnosis was bone in 7 patients (70%) and lung in 3 patients (30%); pituitary, tympanic membrane, liver and brain involvement were also observed. Three (30%) patients had single system involvement and 7 (70%) had multisystem. Four (40%) patients had relapsed and all had multisystemic involvement. The BRAF V600E mutation could be performed in 3 patients. It was negative in two patients and positive in 1 patient. All patients still have remission. Conclusion: Unlike children, LCH has a better course in adults. The most important approach is to determine single or multisystem involvement. In our patients with single system involvement, we obtained response with corticosteroid and surgery alone. We have observed that vinblastin plus metilprednizolon treatment is a good option for multisystemic involvement. In relapsed patients, we obtained a significant response with clofarabine.
Anahtar Kelime: Adult BRAF V600E mutation clofarabin histiocytosis langerhans cell Radiotherapy Treatment vinblastin plus methylprednisolone

Belge Türü: Makale Makale Türü: Araştırma Makalesi Erişim Türü: Erişime Açık
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APA Çelik S, Guven Z, Aşık O, Keklik M, Kaynar L, Eser B, ÇETİN M, ÜNAL A (2023). Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience. , 59 - 64. 10.37990/medr.1159055
Chicago Çelik Serhat,Guven Zeynep Tugba,Aşık Onur,Keklik Muzaffer,Kaynar Leylagul,Eser Bülent,ÇETİN Mustafa,ÜNAL ALİ Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience. (2023): 59 - 64. 10.37990/medr.1159055
MLA Çelik Serhat,Guven Zeynep Tugba,Aşık Onur,Keklik Muzaffer,Kaynar Leylagul,Eser Bülent,ÇETİN Mustafa,ÜNAL ALİ Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience. , 2023, ss.59 - 64. 10.37990/medr.1159055
AMA Çelik S,Guven Z,Aşık O,Keklik M,Kaynar L,Eser B,ÇETİN M,ÜNAL A Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience. . 2023; 59 - 64. 10.37990/medr.1159055
Vancouver Çelik S,Guven Z,Aşık O,Keklik M,Kaynar L,Eser B,ÇETİN M,ÜNAL A Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience. . 2023; 59 - 64. 10.37990/medr.1159055
IEEE Çelik S,Guven Z,Aşık O,Keklik M,Kaynar L,Eser B,ÇETİN M,ÜNAL A "Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience." , ss.59 - 64, 2023. 10.37990/medr.1159055
ISNAD Çelik, Serhat vd. "Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience". (2023), 59-64. https://doi.org/10.37990/medr.1159055
APA Çelik S, Guven Z, Aşık O, Keklik M, Kaynar L, Eser B, ÇETİN M, ÜNAL A (2023). Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience. Medical records-international medical journal (Online), 5(1), 59 - 64. 10.37990/medr.1159055
Chicago Çelik Serhat,Guven Zeynep Tugba,Aşık Onur,Keklik Muzaffer,Kaynar Leylagul,Eser Bülent,ÇETİN Mustafa,ÜNAL ALİ Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience. Medical records-international medical journal (Online) 5, no.1 (2023): 59 - 64. 10.37990/medr.1159055
MLA Çelik Serhat,Guven Zeynep Tugba,Aşık Onur,Keklik Muzaffer,Kaynar Leylagul,Eser Bülent,ÇETİN Mustafa,ÜNAL ALİ Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience. Medical records-international medical journal (Online), vol.5, no.1, 2023, ss.59 - 64. 10.37990/medr.1159055
AMA Çelik S,Guven Z,Aşık O,Keklik M,Kaynar L,Eser B,ÇETİN M,ÜNAL A Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience. Medical records-international medical journal (Online). 2023; 5(1): 59 - 64. 10.37990/medr.1159055
Vancouver Çelik S,Guven Z,Aşık O,Keklik M,Kaynar L,Eser B,ÇETİN M,ÜNAL A Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience. Medical records-international medical journal (Online). 2023; 5(1): 59 - 64. 10.37990/medr.1159055
IEEE Çelik S,Guven Z,Aşık O,Keklik M,Kaynar L,Eser B,ÇETİN M,ÜNAL A "Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience." Medical records-international medical journal (Online), 5, ss.59 - 64, 2023. 10.37990/medr.1159055
ISNAD Çelik, Serhat vd. "Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience". Medical records-international medical journal (Online) 5/1 (2023), 59-64. https://doi.org/10.37990/medr.1159055