دورية أكاديمية
Real-world data on diagnostics, treatment and outcomes of patients with hairy cell leukemia: The HCL-CLLEAR study.
| العنوان: | Real-world data on diagnostics, treatment and outcomes of patients with hairy cell leukemia: The HCL-CLLEAR study. |
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| المؤلفون: | Panovská A; Department of Internal Medicine-Hematology and Oncology, University Hospital and Masaryk University, Brno, Czech Republic., Žák P; 4th Department of Internal Medicine-Hematology, University Hospital and Charles University Faculty of Medicine, Hradec Králové, Czech Republic., Jurková T; Institute of Biostatistics and Analyses, Ltd., Brno, Czech Republic., Arpáš T; Department of Internal Medicine-Hematology and Oncology, University Hospital and Masaryk University, Brno, Czech Republic., Brychtová Y; Department of Internal Medicine-Hematology and Oncology, University Hospital and Masaryk University, Brno, Czech Republic., Vašíková A; Department of Internal Medicine-Hematology and Oncology, University Hospital and Masaryk University, Brno, Czech Republic., Hrabčáková V; Department of Internal Medicine-Hematology and Oncology, University Hospital and Masaryk University, Brno, Czech Republic., Prchlíková A; Department of Internal Medicine-Hematology and Oncology, University Hospital and Masaryk University, Brno, Czech Republic., Filipová M; Department of Internal Medicine-Hematology and Oncology, University Hospital and Masaryk University, Brno, Czech Republic., Doubek M; Department of Internal Medicine-Hematology and Oncology, University Hospital and Masaryk University, Brno, Czech Republic.; Central European Institute of Technology, Masaryk University, Brno, Czech Republic. |
| المصدر: | Hematological oncology [Hematol Oncol] 2024 May; Vol. 42 (3), pp. e3280. |
| نوع المنشور: | Journal Article |
| اللغة: | English |
| بيانات الدورية: | Publisher: Wiley-Blackwell Country of Publication: England NLM ID: 8307268 Publication Model: Print Cited Medium: Internet ISSN: 1099-1069 (Electronic) Linking ISSN: 02780232 NLM ISO Abbreviation: Hematol Oncol Subsets: MEDLINE |
| أسماء مطبوعة: | Original Publication: Oxford, England : Wiley-Blackwell, c1983- |
| مواضيع طبية MeSH: | Leukemia, Hairy Cell*/diagnosis , Leukemia, Hairy Cell*/drug therapy , Leukemia, Hairy Cell*/pathology , Leukemia, Hairy Cell*/mortality , Leukemia, Hairy Cell*/therapy, Humans ; Male ; Female ; Aged ; Middle Aged ; Retrospective Studies ; Adult ; Aged, 80 and over ; Treatment Outcome ; Cladribine/therapeutic use ; Cladribine/administration & dosage ; Follow-Up Studies ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Proto-Oncogene Proteins B-raf/genetics |
| مستخلص: | Hairy cell leukemia (HCL) and HCL-like disorders have to be distinguished because of their different biology and treatment response. Thus, we conducted a retrospective study on patients with HCL and hairy cell leukemia variant (HCLv) to assess diagnostic algorithms and treatment outcomes in a real-world setting. We analyzed 225 HCL and 26 HCLv patients with median follow-up of 67.9 months (HCL) and 20.1 months (HCLv). Median age at diagnosis was 56.2 (HCL) and 69.5 years (HCLv), male predominance was observed in both groups (76.0% vs. 73.1%). Diagnostics was mostly based on morphological evidence of hairy cells in the peripheral blood and bone marrow. At diagnosis, BRAF V600E mutation was detected in 94.7% of examined HCL patients and in no HCLv patient. Front-line treatment was indicated in 205 (91.1%) HCL and 18 (69.2%) HCLv patients. The majority of HCL patients were administered a cladribine-based regimen (91.2%). Overall response rate (ORR) was higher in cladribine-treated patients compared to those given other treatments (97.7% vs. 81.3%), the same applied with achieving Complete remission (CR) (91.2% vs. 62.5%). HCLv treatment was heterogeneous, but cladribine remained the most frequent option (44.4%) with ORR 81.3% and CR rates 43.8%. Second-line treatment was indicated in 52 HCL and 8 HCLv patients, 25.4% and 44.4% of those treated in first-line. In the whole HCL group, median time to next treatment (TTNT) was not reached and 10-year TTNT was estimated at 74.1%. HCLv patients who underwent first-line treatment had a median TTNT of 56 months. The median overall survival (OS) in HCL patients was not reached compared to HCLv with a median OS of 9.5 years. These data confirm an excellent prognosis for HCL patients treated with cladribine-based therapy. On the contrary, HCLv with its aggressive behavior represents a group of patients in whom novel treatment approaches are needed. (© 2024 The Authors. Hematological Oncology published by John Wiley & Sons Ltd.) |
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| معلومات مُعتمدة: | EARTRIS-CZ; LX22NPO5102 National Institute for Cancer Research (Program EXCELES); Czech CLL Study Group |
| فهرسة مساهمة: | Keywords: BRAF V600Emutation; cladribine; hairy cell leukemia; hairy cell leukemia variant; overall survival; time to next treatment |
| المشرفين على المادة: | 47M74X9YT5 (Cladribine) EC 2.7.11.1 (Proto-Oncogene Proteins B-raf) |
| تواريخ الأحداث: | Date Created: 20240514 Date Completed: 20240514 Latest Revision: 20240514 |
| رمز التحديث: | 20240514 |
| DOI: | 10.1002/hon.3280 |
| PMID: | 38741354 |
| قاعدة البيانات: | MEDLINE |
Full Text Finder | تدمد: | 1099-1069 |
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| DOI: | 10.1002/hon.3280 |