Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience

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dc.contributor.authorÇelik, Serhat
dc.contributor.authorGüven, Zeynep Tuğba
dc.contributor.authorAşık, Onur
dc.contributor.authorKeklik, Muzaffer
dc.contributor.authorKaynar, Leylagül
dc.contributor.authorEser, Bülent
dc.contributor.authorÇetin, Mustafa
dc.date.accessioned2025-01-21T16:13:46Z
dc.date.available2025-01-21T16:13:46Z
dc.date.issued2023
dc.departmentKırıkkale Üniversitesi
dc.description.abstractAim: 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.
dc.identifier.doi10.37990/medr.1159055
dc.identifier.endpage64
dc.identifier.issn2687-4555
dc.identifier.issue1
dc.identifier.startpage59
dc.identifier.trdizinid1196642
dc.identifier.urihttps://doi.org/10.37990/medr.1159055
dc.identifier.urihttps://search.trdizin.gov.tr/tr/yayin/detay/1196642
dc.identifier.urihttps://hdl.handle.net/20.500.12587/22002
dc.identifier.volume5
dc.indekslendigikaynakTR-Dizin
dc.language.isoen
dc.relation.ispartofMedical records-international medical journal (Online)
dc.relation.publicationcategoryMakale - Ulusal Hakemli Dergi - Kurum Öğretim Elemanı
dc.rightsinfo:eu-repo/semantics/openAccess
dc.snmzKA_20241229
dc.subjectMikrobiyoloji
dc.subjectOnkoloji
dc.titleAdult Onset Langerhans Cell Histiocytosis: A Single Center Experience
dc.typeArticle

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