Wang Anna, Guo Wei, Damiani Daniela, Sumbly Vikram, Goyal Gaurav, Du Zhonghua, Bai Ou
Department of Hematology, Tumor Center, The First Hospital of Jilin University, Changchun, China.
Division of Hematology and Stem Cell Transplantation, Udine Hospital, Udine, Italy.
Transl Cancer Res. 2023 Jul 31;12(7):1873-1882. doi: 10.21037/tcr-23-828. Epub 2023 Jul 20.
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/ 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.
B 细胞原淋巴细胞白血病（B-PLL）是一种罕见的成熟 B 细胞肿瘤，临床病程侵袭性强，预后较差。其特征为显著脾肿大，血液中原淋巴细胞超过淋巴细胞的 55%。基于嘌呤类似物的化疗免疫疗法是 B-PLL 的一线治疗方法。由于其罕见性，关于苯达莫司汀和利妥昔单抗（BR）方案疗效的报道较少。我们的研究展示了 3 例 BR 方案治疗 B-PLL 有效的病例，并为临床治疗提供经验。
本报告描述了 3 例男性患者（中位年龄：66 岁）的病例，他们最初表现为腹部不适。体格检查和影像学检查显示脾肿大，而外周血涂片显示原淋巴细胞计数超过淋巴细胞的 70%。因此，这 3 例患者被诊断为 B-PLL。进一步的分子检测显示，他们存在与对传统化疗耐药相关的 P53 异常（17p 缺失/突变）。此外，其中 1 例患者具有高度复杂的核型和多个基因突变。所有患者均接受了 4 个周期的 BR 方案治疗，其中 2 例患者还接受了 2 个周期的利妥昔单抗单药治疗。最终，患者分别实现了持续 25、33 和 34 个月的完全缓解（CR），中位随访时间为 34 个月。BR 方案的不良事件主要包括 3 级血液学毒性。而且，该治疗耐受性良好。
本病例系列表明，BR 方案有望使 B-PLL 患者获得深度缓解。仍需要进行前瞻性试验以进一步阐明。
