B细胞前淋巴细胞白血病（B-PLL）是一种罕见的成熟B细胞肿瘤，具有侵袭性临床进程和不良预后。其特征是脾肿大显著以及血液中超过55%的淋巴细胞为前淋巴细胞。嘌呤类似物为B-PLL的一线疗法。由于其罕见性，关于苯达莫司汀和利妥昔单抗（BR）方案疗效的报道很少。我们的研究报道了三例BR在B-PLL治疗中的有效性，并为临床治疗提供了经验。本报告描述了三名男性患者的病例（中位年龄：66岁），最初表现为腹部不适。体格检查和影像学显示脾肿大，外周血涂片显示前淋巴细胞计数超过淋巴细胞的70%。因此，三名患者被诊断为B-PLL。进一步的分子检测显示他们携带P53异常（17p缺失/TP53突变），与传统化疗的耐药性有关。此外，其中一例患者有高度复杂的核型和多基因突变。所有患者接受了四个疗程的BR治疗，其中两例还接受了两个额外疗程的利妥昔单抗单药治疗。最终，患者获得了完全缓解（CR），分别持续了25、33和34个月，随访中位时间为34个月。BR的不良事件主要包括3级造血毒性，治疗耐受性良好。这个病例系列表明BR方案对B-PLL患者达到深度缓解的前景很有希望。为了进一步阐明，仍需要进行前瞻性试验。2023翻译癌症研究。版权所有。

B-cell prolymphocytic leukemia (B-PLL) is a rare mature B-cell tumor with an aggressive clinical course and poor prognosis. It is characterized by prominent splenomegaly and prolymphocytes exceeding 55% of the lymphoid cells in the blood. Purine analog-based chemo-immunotherapy is the first-line therapy for B-PLL. Owing to its rarity, there are few reports on the efficacy of bendamustine and rituximab (BR) regimen. Our study presents three cases of BR being effective in the treatment of B-PLL and provides experience for clinical treatment.This report describes the cases of three male patients (median age: 66 years old) who initially presented with abdominal discomfort. Physical examinations and imaging revealed splenomegaly, while a peripheral blood (PB) smear revealed a prolymphocyte count exceeding 70% of the lymphoid cells. Therefore, the three patients were diagnosed with B-PLL. Further molecular detection showed that they harbored P53 abnormalities (17p deletion/TP53 mutation) associated with resistance to conventional chemotherapies. In addition, one of the patients had a highly complex karyotype and multiple gene mutations. All patients underwent four cycles of BR, and two of them received two further cycles of rituximab monotherapy. Ultimately, the patients achieved a complete response (CR) that lasted for 25, 33, and 34 months, respectively, with a median follow-up time of 34 months. The adverse events of the BR mainly included a grade 3 haematological toxicities. Also, the treatment was well-tolerated.This case series suggests that BR regimen is promising for bringing deep remission to patients with B-PLL. Prospective trials are still required for further elucidation.2023 Translational Cancer Research. All rights reserved.