TY  - JOUR
TI  - Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience
AB  - 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.
AU  - Çelik, Serhat
AU  - ÇETİN, Mustafa
AU  - ÜNAL, ALİ
AU  - Guven, Zeynep Tugba
AU  - Aşık, Onur
AU  - Keklik, Muzaffer
AU  - Kaynar, Leylagul
AU  - Eser, Bülent
DO  - 10.37990/medr.1159055
PY  - 2023
JO  - Medical records-international medical journal (Online)
VL  - 5
IS  - 1
SN  - 2687-4555
SP  - 59
EP  - 64
DB  - TRDizin
UR  - http://search/yayin/detay/1196642
ER  -