Adult Onset Langerhans Cell Histiocytosis: A Single Center Experience

نویسندگان

چکیده

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

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ژورنال

عنوان ژورنال: Medical records-international medical journal

سال: 2023

ISSN: ['2687-4555']

DOI: https://doi.org/10.37990/medr.1159055