Clinical and biological characteristics of non-IgM lymphoplasmacytic lymphoma
10.3760/cma.j.issn.0253-2727.2022.07.007
- VernacularTitle:非IgM型淋巴浆细胞淋巴瘤临床及生物学特征
- Author:
Ying YU
1
;
Wenjie XIONG
;
Jiawen CHEN
;
Yang JIAO
;
Yuting YAN
;
Qi WANG
;
Dehui ZOU
;
Wei LIU
;
Huimin LIU
;
Rui LYU
;
Lugui QIU
;
Shuhua YI
Author Information
1. 中国医学科学院血液病医院（中国医学科学院血液学研究所），实验血液学国家重点实验室，国家血液系统疾病临床医学研究中心，细胞生态海河实验室，天津 300020
- Keywords:
Lymphoplasmacytic lymphoma;
Immunoglobulin G;
Immunoglobulin A;
Clinical characteristics;
Cytogenetics
- From:
Chinese Journal of Hematology
2022;43(7):568-574
- CountryChina
- Language:Chinese
-
Abstract:
Objective:The study aims to explore the clinical and biological characteristics of patients with non-IgM lymphoplasmacytic lymphoma (LPL) .Methods:The clinical data of 340 patients with LPL admitted to the Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College were collected retrospectively, including 23 cases of the non-IgM LPL and 317 cases of the Waldenstr?m's macroglobulinemia (WM) , from July 1993 to August 2020. The clinical and biological characteristics of the two groups were compared.Results:Among 23 patients with the non-IgM type LPL, two patients secreted monoclonal IgA, 14 patients secreted monoclonal IgG, and seven patients did not secrete monoclonal immunoglobulin. The median age of the non-IgM LPL and WM were both 62 (35-81) years old. Compared with the WM group, the proportion of women (56.5% vs 27.3%, P=0.007) , the proportion of splenomegaly (60.1% vs 43.8%, P=0.100) , and the proportion of extranodal invasion (21.7% vs 12.3%, P=0.672) in non-IgM LPL group were higher. Eighteen patients were tested for MYD88 gene mutation, and the overall mutation rate of MYD88 was 55.6%. In the non-IgM LPL group, a total of 17 patients received treatment, which had a comparable proportion (94.4% vs 92.7%, P=0.488) to the WM group. Sixteen patients were evaluated for efficacy, and the overall remission rate of the first-line treatment was 87.5%. The median follow-up time was 33.9 (3.5-125.1) months, and the median PFS and OS were both not reached. The 3-year PFS and OS rates were 71.4% and 68.9%, respectively. In the WM group, the median PFS was 66.2 months and the median OS was 78.1 months. Compared with the WM group, in the non-IgM group no significant differences in PFS ( P=0.340) and OS ( P=0.544) were seen. Conclusion:The clinical and biological characteristics of the non-IgM LPL and WM patients were similar. However, the proportion of women and extranodal involvement were higher in the non-IgM LPL group. The survival and prognosis of the non-IgM LPL patients were similar to those of the WM patients.
