Lymphoplasmacytic lymphoma: clinical analysis of 33 cases / 白血病·淋巴瘤

ABSTRACT

Objective:To investigate the clinicopathological characteristics and survival of patients with lymphoplasmacytic lymphoma (LPL).Methods:The data of 33 newly diagnosed LPL patients in the First Affiliated Hospital of Dalian Medical University from July 2003 to May 2021 were retrospectively analyzed. The clinical characteristics, bone marrow cell morphology, immunophenotyping, chromosomal karyotype, gene mutation, treatment response and prognosis were analyzed, and Kaplan-Meier method was used to analyze the survival of patients.Results:The median age of onset of 33 patients was 66 years old (55-84 years old). There were 26 males (78.8%) and 7 females (21.2%). The common clinical manifestations were anemia (31 cases, 93.9%), enlarged lymph nodes (16 cases, 48.5%) and B symptoms (8 cases, 24.2%). All patients had bone marrow involvement and M protein, 23 of them (69.7%) were type IgM-κ, 8 cases (24.2%) were type IgM-λ, 1 case (3.0%) was type IgG-κ, and 1 case (3.0%) was type IgA-κ. Lymphocytes, lymphoplasmacytes or plasma cells was increased in bone marrow smear; 22 patients underwent immunophenotyping of bone marrow by flow cytometry, and all patients expressed B cell surface antigens (CD19 and CD20), 16 patients (72.7%) lost the expression of CD5 and CD10, 13 patients (59.1%) expressed or weakly expressed CD138 and 5 patients (22.7%) expressed CD38. Seven out of 23 cases (30.4%) who received chromosome examination had abnormal chromosomal karyotype. Fourteen out of 16 cases (87.5%) who received MYD88 L265P mutation detection harbored the mutation. Among 21 patients with evaluable efficacy, 18 patients (85.7%) responded to treatment, achieving partial remission or stable disease, but the rate of complete remission was low (14.3%, 3/21). The median follow-up time was 34 months (2-102 months), 1 case was lost. The median overall survival time was not reached, and the 3-year and 5-year overall survival rates were 79.2% and 67.9%, respectively.Conclusions:LPL is a rare indolent small B-cell lymphoma with a long course and a variety of manifestations, which is commonly seen in elderly men.Serological examination, bone marrow cell morphology and biopsy, immunophenotyping and MYD88 L265P mutation detection are important for the diagnosis and differential diagnosis.