PDF(Pubmed)
Abstract：
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
摘要：
■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，具有侵袭性临床病程和不良预后。它的特征是明显的脾肿大和前淋巴细胞超过血液中淋巴样细胞的55％。基于嘌呤类似物的化学免疫疗法是B-PLL的一线疗法。由于它的稀有性,关于苯达莫司汀和利妥昔单抗(BR)方案疗效的报道很少.我们的研究提出了3例BR在B-PLL治疗中有效，并为临床治疗提供了经验。
■本报告描述了三名男性患者（中位年龄：66岁）最初出现腹部不适的病例。体格检查和影像学显示脾肿大，而外周血（PB）涂片显示前淋巴细胞计数超过淋巴样细胞的70％。因此,3例患者被诊断为B-PLL。进一步的分子检测表明，它们具有与对常规化学疗法的抗性相关的P53异常（17p缺失/TP53突变）。此外,其中一名患者具有高度复杂的核型和多个基因突变.所有患者都接受了四个周期的BR，其中两人又接受了两个周期的利妥昔单抗单药治疗.最终,患者获得了持续25、33和34个月的完全缓解（CR），分别,中位随访时间为34个月。BR的不良事件主要包括3级血液毒性。此外，治疗耐受性良好.
■本系列病例表明BR方案有望为B-PLL患者带来深度缓解。仍需进行前瞻性试验以进一步阐明。
■本报告描述了三名男性患者（中位年龄：66岁）最初出现腹部不适的病例。体格检查和影像学显示脾肿大，而外周血（PB）涂片显示前淋巴细胞计数超过淋巴样细胞的70％。因此,3例患者被诊断为B-PLL。进一步的分子检测表明，它们具有与对常规化学疗法的抗性相关的P53异常（17p缺失/TP53突变）。此外,其中一名患者具有高度复杂的核型和多个基因突变.所有患者都接受了四个周期的BR，其中两人又接受了两个周期的利妥昔单抗单药治疗.最终,患者获得了持续25、33和34个月的完全缓解（CR），分别,中位随访时间为34个月。BR的不良事件主要包括3级血液毒性。此外，治疗耐受性良好.
■本系列病例表明BR方案有望为B-PLL患者带来深度缓解。仍需进行前瞻性试验以进一步阐明。
