Clinical analysis of prolymphoblastic leukemia: the rare hematological malignancy in the elderly.

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.

Conclusion: 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.