根据GMALL B-ALL/NHL 2002方案治疗伯基特淋巴瘤和伯基特白血病结果的真实世界数据:三级中心经验。

IF 2.7 4区 医学 Q2 HEMATOLOGY
Ricardo Kosch, Christoph Schaefers, Christian Frenzel, Walter Fiedler, Katja Weisel, Carsten Bokemeyer, Christoph Seidel, Finn-Ole Paulsen
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引用次数: 0

摘要

伯基特淋巴瘤(BL)或白血病是一种高度侵袭性的b细胞恶性肿瘤,需要强化治疗。尽管出现了各种不同的方案,但很少有实际数据存在于GMALL B-ALL/NHL 2002方案中。由于这种疾病的罕见性,比较分析仍然具有挑战性。方法:这项回顾性研究检查了在汉堡-埃本多夫大学医学中心(2012-2022)接受GMALL方案的组织学证实的BL成年患者(pts)的总缓解率(ORR)、无进展生存期(PFS)、总生存期(OS)和毒性。统计分析确定了影响结果的预后因素。结果:共纳入48例患者(中位年龄51.5岁,范围22-78)。多数表现为Ann Arbor IV期(68.7%)和BL-IPI高危疾病(54.1%)。伯基特白血病占27.1%,HIV阳性占27%,中枢神经系统受累占18%。ORR为81.6%(完全缓解73.5%,部分缓解8.1%)。中位随访38个月(0-103)后,中位PFS和OS均未达到;PFS和OS的3年生存率均为73%。虽然没有在多变量分析中得到证实,但伯基特白血病(HR 3.86, P = 0.021)和任何骨髓受累(HR 3.80, P = 0.049)成为不良预后指标。严重粘膜炎(≥3级)是常见的(91.7%),87.5%至少经历过一次发热性中性粒细胞减少发作。未观察到5级治疗相关毒性。结论:这些现实世界的研究结果强调了GMALL方案在BL管理中的有效性,其结果与现有方案相当,但毒性高。进一步的比较试验是确定最佳治疗策略的必要条件。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Real-World Data on Outcome of Burkitt Lymphoma and Burkitt Leukemia Treated According to the GMALL B-ALL/NHL 2002 Protocol: A Tertiary Center Experience.

Introduction: Burkitt lymphoma (BL) or leukemia represents a highly aggressive B-cell malignancy requiring intense therapy. Although various different protocols have emerged, few real-world data exist for the GMALL B-ALL/NHL 2002 regimen. Comparative analyses remain challenging because of the disease's rarity.

Methods: This retrospective study examined the overall response rate (ORR), progression-free survival (PFS), overall survival (OS), and toxicity in adult patients (pts) with histologically confirmed BL who received the GMALL protocol at the University Medical Center Hamburg-Eppendorf (2012-2022). Statistical analysis identified prognostic factors affecting outcomes.

Results: A total of 48 pts (median age 51.5 years, range 22-78) were included. Most presented with Ann Arbor stage IV (68.7%) and high-risk disease by the BL-IPI (54.1%). Burkitt leukemia was present in 27.1%, HIV positivity in 27%, and CNS involvement in 18%. The ORR was 81.6% (73.5% complete response, 8.1% partial response). After a median follow-up of 38 months (range 0-103), the median PFS and OS were not reached; 3-year rates for both PFS and OS were 73%. While not confirmed in multivariate analysis, Burkitt leukemia (HR 3.86, P = .021) and any bone marrow involvement (HR 3.80, P = .049) emerged as adverse prognostic indicators. Severe mucositis (grade ≥ 3) was common (91.7%), and 87.5% experienced at least one febrile neutropenia episode. No grade 5 treatment-related toxicity was observed.

Conclusion: These real-world findings underscore the efficacy of the GMALL protocol in BL management, with outcomes comparable to established regimens but coupled with high toxicity rates. Further comparative trials are essential to define the optimal therapeutic strategy.

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来源期刊
CiteScore
2.70
自引率
3.70%
发文量
1606
审稿时长
26 days
期刊介绍: Clinical Lymphoma, Myeloma & Leukemia is a peer-reviewed monthly journal that publishes original articles describing various aspects of clinical and translational research of lymphoma, myeloma and leukemia. Clinical Lymphoma, Myeloma & Leukemia is devoted to articles on detection, diagnosis, prevention, and treatment of lymphoma, myeloma, leukemia and related disorders including macroglobulinemia, amyloidosis, and plasma-cell dyscrasias. The main emphasis is on recent scientific developments in all areas related to lymphoma, myeloma and leukemia. Specific areas of interest include clinical research and mechanistic approaches; drug sensitivity and resistance; gene and antisense therapy; pathology, markers, and prognostic indicators; chemoprevention strategies; multimodality therapy; and integration of various approaches.
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