Adult Onset Langerhans Cell Histiocytosis: Clinical Characteristics and Treatment Outcomes

被引:1
作者
Arslan Davulcu E. [1 ]
Soyer N. [2 ]
Demirci Z. [2 ]
Güneş A. [2 ]
Vural F. [2 ]
Şahin F. [2 ]
Töbü M. [2 ]
Kamer S. [3 ]
Hekimgil M. [4 ]
Saydam G. [2 ]
机构
[1] University of Health Sciences Bakırkoy Dr. Sadi Konuk Training and Research Hospital, Hematology Clinic, Zuhuratbaba Mah, Dr. Tevfik Sağlam Cad No:11, Bakırköy, Istanbul
[2] Faculty of Medicine, Hematology Department, Ege University, İzmir
[3] Faculty of Medicine, Radiation Oncology Department, Ege University, İzmir
[4] Faculty of Medicine, Pathology Department, Ege University, İzmir
关键词
Cladribine; Histiocytic disorders; Langerhans cell histiocytosis;
D O I
10.1007/s44228-023-00034-w
中图分类号
学科分类号
摘要
Purpose: Langerhans cell histiocytosis (LCH) is a rare disease that can affect all tissues and organs. Our study evaluated the clinical characteristics and treatment outcomes of adult-onset LCH patients in a tertiary center. Materials and Methods: Adult patients diagnosed with LCH were retrospectively evaluated. Their initial symptoms, stratification according to disease involvement, treatment details, treatment responses, and overall and progression-free survival (PFS) were analyzed. Results: Thirty-three patients were included. There were 21 single system LCH, 10 multisystem LCH, and 2 pulmonary LCH patients. Patients with single system unifocal involvement were successfully treated with local therapies such as surgery and radiotherapy. Most of the multisystem LCH patients and patients with single system multifocal involvement were treated with systemic chemotherapy. Cladribine was the first choice in 10 out of 11 patients who received chemotherapy. Among all patients, the overall response rate (ORR) was 97%. Among those who had cladribine in the first-line the ORR was 81%. All these patients achieved a complete remission and were alive at the last visit. The median follow-up was 38 (range, 2–183) months. The median PFS has not yet been reached. Ten-year PFS was 90.9%. Conclusion: Besides successful local treatments with surgery and radiotherapy, our study provides information for front-line cladribine treatment. © 2023, The Author(s).
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页码:101 / 106
页数:5
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