Lymphoplasmacytic lymphoma/Waldenstr?m macroglobulinemia: a clinical analysis of 13 cases
10.3760/cma.j.cn115356-20200425-00106
- VernacularTitle:淋巴浆细胞淋巴瘤/华氏巨球蛋白血症13例临床分析
- Author:
Yingying WANG
1
;
Liang SHAO
;
Yi ZHOU
;
Minghui LIU
;
Bei XIONG
;
Jiang WU
;
Li HE
;
Hui XIAO
;
Xuelan ZUO
;
Fei CHEN
;
Fuling ZHOU
;
Shangqin LIU
Author Information
1. 武汉大学中南医院血液内科 430071
- From:
Journal of Leukemia & Lymphoma
2020;29(10):590-594
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical characteristics of patients with lymphoplasmacytic lymphoma/Waldenstr?m macroglobulinemia (LPL/WM), and the diagnosis and optimal treatment of LPL/WM.Methods:The clinical data of 13 LPL/WM patients treated in Zhongnan Hospital of Wuhan University from January 2013 to June 2018 were retrospectively analyzed, and the literature was reviewed.Results:The median age of 13 patients was 60 years old (35-79 years old). There were 12 males and 1 female. Initial symptom was fatigue or edema of both lower limbs for majority of patients. All patients had immunoglobulin M (IgM) monoclonal, 3 of them had elevated immunoglobulin G (IgG) level, including 1 patient with monoclonal IgG. LDH was increased in 2 patients. Coombs test was positive in 5 patients. MyD88 gene mutation status was detected in 8 patients, of which gene mutation in 5 patients was positive. Among 13 patients, 1 patient lost follow-up, 3 patients died, 9 patients were alive with the median survival of 36 months (19-81 months).Conclusions:Incidence of LPL/WM is relatively low with a generally indolent evolution, but heterogeneity is not negligible. Few patients have poor treatment response with a quick disease progress. The high-risk patients undergoing hematopoietic stem cell transplantation after remission-induction chemotherapy may improve the prognosis.