淋巴浆细胞淋巴瘤/华氏巨球蛋白血症13例临床分析

Lymphoplasmacytic lymphoma/Waldenström macroglobulinemia:a clinical analysis of 13 cases

目的探讨淋巴浆细胞淋巴瘤/华氏巨球蛋白血症(LPL/WM)患者的临床特征及其诊断与治疗。方法回顾性分析武汉大学中南医院2013年1月至2018年6月收治的13例LPL/WM患者的临床资料,并进行文献复习。结果13例LPL/WM患者中位年龄60岁(35~79岁),男性12例,女性1例,多以乏力或双下肢水肿为首发症状。患者均存在单克隆免疫球蛋白M(IgM),3例伴有免疫球蛋白G(IgG)升高,其中1例为单克隆IgG;乳酸脱氢酶升高2例;5例患者直接抗人球蛋白试验阳性。8例患者检测了MyD88基因突变情况,其中5例突变阳性。13例患者中,1例失访,3例死亡,9例生存,中位生存时间36个月(19~81个月)。结论LPL/WM发病率较低,多数进展缓慢,但异质性较强。少数患者进展快,治疗效果差。高危患者诱导化疗缓解后行造血干细胞移植可能改善预后。

To investigate the clinical characteristics of patients with lymphoplasmacytic lymphoma/Waldenström 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.