目的:探讨和分析罕见的淋巴细胞白血病(PLL)的临床和预后特点,以期为PLL的临床诊治和基础研究提供新的参考。
方法:收集福建医科大学附属协和医院血液科2011年1月1日至2023年5月31日收治的8例PLL患者的临床资料。并对其临床治疗及预后进行分析。同时,检索了PubMed的最新文献,系统地讨论了PLL诊断和治疗的研究进展。
结果:在这项研究中,在8名PLL患者中,男性6人,女性2人;年龄从52岁到80岁,平均年龄为70.5岁。免疫表型分为B-PLL(7例)和T-PLL(1例)。形态学,流式细胞仪,所有患者均进行骨髓细胞遗传学和分子生物学检查。其中,1例确诊后拒绝化疗,短时间内死亡,其他7例接受标准化疗。在7名患者中,一名患者因化疗后骨髓抑制导致严重感染死亡,1例患者在化疗后3个月内死亡;2例患者在化疗后4个月和7个月死于进展.共有3例患者化疗后完全缓解(CR),1例患者接受异基因造血干细胞移植,无疾病进展或复发.
结论:PLL是一种罕见的淋巴恶性肿瘤,预后极差,这往往发生在老年人身上,PLL的临床表现缺乏特异性。化疗联合靶向药物或表观遗传药物可能使PLL患者受益。应在获得CR后进行造血干细胞移植,以最大程度地改善预后。
OBJECTIVE: To discuss and analyze the clinical and prognostic characteristics of rare prolymphocytic leukemia (PLL), in order to provide new references for the clinical diagnosis and treatment and basic research of PLL.
METHODS: The clinical data of 8 patients with PLL admitted to the Department of Hematology in Fujian Medical University Union Hospital from January 1, 2011 to May 31, 2023 were collected and retrospectively studied, and the clinical treatment and prognosis were analyzed. Meanwhile, the latest literature from PubMed was retrieved to systematically discuss the research progress in the diagnosis and treatment of PLL.
RESULTS: In this study, of the 8 patients with PLL, 6 were males and 2 were females; the ages ranged from 52 to 80 years, with a median age of 70.5 years. The immunophenotypes were divided into B-PLL (7 cases) and T-PLL (1 case). Morphological, flow cytometric, cytogenetic and molecular biological tests of bone marrow cells were performed in all patients. Among them, 1 case refused chemotherapy after diagnosis and died in a short time, the other 7 cases received standard chemotherapy. Among the 7 patients, one patient died of severe infection caused by myelosuppression after chemotherapy, one patient died within 3 months after chemotherapy; two patients died of progression at 4 and 7 months after chemotherapy. A total of 3 patients achieved complete remission (CR) after chemotherapy, and 1 patient underwent allogeneic hematopoietic stem cell transplantation without disease progression or recurrence.
CONCLUSIONS: PLL is a rare lymphoid malignancy with an extremely poor prognosis, which tends to occur in the elderly, and the clinical manifestations of PLL lack specificity. Chemotherapy combined with targeted drugs or epigenetic drugs may benefit PLL patients. Hematopoietic stem cell transplantation should be performed after CR is obtained to improve the prognosis to the greatest extent.