Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience

Authors : Serhat ÇELİK, Zeynep Tuğba GÜVEN, Onur AŞIK, Muzaffer KEKLİK, Leylagül KAYNAR, Bülent ESER, Mustafa ÇETİN, Ali ÜNAL

Pages : 59-64

Doi:10.37990/medr.1159055

View : 19 | Download : 4

Publication Date : 2023-01-15

Article Type : Research Paper

Abstract :Aim: Langerhans cell histiocytosis insert ignore into journalissuearticles values(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 insert ignore into journalissuearticles values(80%); of them were male and 2 insert ignore into journalissuearticles values(20%); were female. The median age of the patients was 34.5 ± 8.4 insert ignore into journalissuearticles values(23-52); years. Although the most common involvement at the time of diagnosis was bone in 7 patients insert ignore into journalissuearticles values(70%); and lung in 3 patients insert ignore into journalissuearticles values(30%);; pituitary, tympanic membrane, liver and brain involvement were also observed. Three insert ignore into journalissuearticles values(30%); patients had single system involvement and 7 insert ignore into journalissuearticles values(70%); had multisystem. Four insert ignore into journalissuearticles values(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.
Keywords : Adult, BRAF V600E mutation, clofarabin, histiocytosis, langerhans cell, Radiotherapy, Treatment, vinblastin plus methylprednisolone, Adult, BRAF V600E mutation, clofarabin, histiocytosis, langerhans cell, Radiotherapy, Treatment, vinblastin plus methylprednisolon