614 | LONG TERM FOLLOW‐UP OF PATIENTS WITH MANTLE CELL LYMPHOMA, WHO UNDERWENT HIGH‐DOSE CHEMOTHERAPY AND AUTOLOGOUS HEMATOPOIETIC CELL TRANSPLANTATION IN FIRST REMISSION
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Introduction: Mantle cell lymphoma (MCL) is a rare, aggressive, and incurable disease, although survival has improved in recent years.Data on localized stages are scarce, and the optimal treatment approach remains unclear.This study aimed to analyze the clinical characteristics, treatment strategies, and prognosis of patients with localized MCL.Patients and methods: This retrospective study included patients diagnosed with localized MCL, Ann-Arbor stage I-II, between January 1995 and May 2022 in different Spanish centers.Clinical and biological variables at diagnosis, treatment regimens, response rates and survival outcomes were collected.Results: A total of 48 patients with localized MCL were identified, of whom 23 (48%) underwent staging by PET/CT.All patients had a bone marrow biopsy at diagnosis, and none showed bone marrow infiltration.The baseline characteristics at diagnosis were as follows: median age of 66.6 years (range: 56.4-74.8),36 males (75%), stage II in 29 patients (60%), bulky disease (> 5 cm) in 5 (10%), extranodal involvement in 33 (69%), with 70% occurring in the Waldeyer's ring, and high-risk MIPI in 21patients (43.7%).Regarding biological characteristics, 13 cases (29%) were classified as the pleomorphic/blastoid variant, Ki-67 > 30% was observed in24 (58%), and TP53 overexpression was found in 7 out of 25 patients (30%).First-line treatment was administered to 46 patients (96%): chemotherapy (CT) � radiotherapy (RT) in 38 (82%) and RT alone in 6 (13%).The CT regimens used included: intensive CT in 13 patients (34%) (R-CHOP/R-DHAP: 6,R-HyperCVAD: 5,R-EPOCH: 1,R-CHOP/R-ESHAP:1), intermediate-intensity CT in 20 (53%) (R-CHOP: 12, R-Bendamustine: 2,VR-CAP: 4,CHOP:2), andlow-intensity/-palliativeCTin5 (13%) (Chlorambucil: 2, CVP:2, CNOP:1).8patients (17%) underwent autologous stem cell transplantation (ASCT), and 10 (22%) received rituximab maintenance therapy.Response rates were as follows: complete response (CR) in 40 patients (87%), CR with incomplete recovery (CRi) in 2 (4.3%), partial response (PR) in 2 (4.3%), and progression in 2 (4.3%).With a median follow-up of 6.5 years (range: 2.6-9.1) for surviving patients, 22 (48%) relapsed, with 12 (67%) relapsing at stage I-II.The median time to relapse was 47.8 months (range: 45.9-53.8).The median progression-free survival (PFS) was 4.4 years (95% CI: 2.5-10), and the median overall survival (OS) was 11.6 years (95% CI: 5.5-15.1).In the univariate analysis of prognostic factors for PFS, age > 65 years (p = 0.04, 95% CI: 1.3-11.8)and Ki-67 > 30% (p = 0.04, 95% CI: 0.23-1.96)were associated with a worse prognosis.Conclusions: Approximately 70% of localized LCMs are diagnosed in Waldeyer's ring.A high proportion present with highrisk histologic variants and high proliferation rates.Treatments approaches are highly heterogeneous, and often include radiotherapy.However, survival curves do not show a plateau, indicating that despite having localized disease, these patients are not cured of their disease.
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Contrôle bibliographique ouvert
DOI retrouvé dans Crossref DOI retrouvé ; titre concordant.
- Titre Crossref
- 614 | LONG TERM FOLLOW‐UP OF PATIENTS WITH MANTLE CELL LYMPHOMA, WHO UNDERWENT HIGH‐DOSE CHEMOTHERAPY AND AUTOLOGOUS HEMATOPOIETIC CELL TRANSPLANTATION IN FIRST REMISSION
- Date Crossref
- 01/06/2025
- Éditeur
- Wiley
- Type
- journal-article
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