24例华氏巨球蛋白血症临床分析

目的 华氏巨球蛋白血症(Waldenstr(o)m's macroglobulinemia,WM)是一种特殊类型的非霍奇金淋巴瘤,其发病率极低,国内外相关数据较为缺乏.本研究旨在探讨WM的临床特点、预后因素及诊治方法. 方法 回顾性分析2011-01-01-2016-01-01郑州大学人民医院诊治的24例WM患者临床资料. 结果 24例WM患者,男16例,女8例,男女比例为2∶1,年龄42~79岁,中位年龄62.5岁;贫血(20例,83.3%)是最常见的临床表现,中位血红蛋白水平75(46~145) g/L;中位IgM水平32.9(6.4~79.3) g/L,其中κ型18例(75%),λ型6例(25%).16例患者行流式细胞术检测,13例(81.3%)表现为sIgM+CD5-CD10-CD19+CD20+CD22+CD23-.全组中位无进展生存时间(PFS)为7.5(1~51)个月.单因素分析结果显示,年龄、血红蛋白(Hb)、血小板(PLT)、β2微球蛋白(β2-MG)、IgM水平、白蛋白(ALB)、血清肌酐(SCr)、乳酸脱氢酶(LDH)、C反应蛋白(CRP)和合并重度免疫不全麻痹影响患者PFS,应用含有利妥昔单抗或硼替佐米的化疗方案组PFS相对较长.多因素分析结果显示,年龄(P=0.008)、IgM水平(P=0.028)和SCr(P=0.005)与预后相关. 结论 WM好发于老年男性,以IgM κ型多见,具有惰性B细胞淋巴瘤的特点,年龄、IgM水平和SCr是影响WM预后的独立危险因素,利妥昔单抗或硼替佐米的应用有望延长PFS.

Clinical features of 24 cases of Waldenstr(o)m's macroglobulinemia

OBJECTIVE Waldenstr(o)m's macroglobulinemia(WM) is a special type of non-Hodgkin's lymphoma.Its incidence is very low.Domestic and international relevant data is relatively lack.The study was to investigate the clinical manifestations, diagnosis, therapy and prognosis of WM.METHODS Clinical data of 24 patients with WM in Zhengzhou University People's Hospital from January 1, 2011 to January 1, 2016 were analyzed retrospectively.RESULTS Among the 24 cases, the ratio of male to female was 2∶1(16 males and 8 females).The median age was 62.5(42-79) years old.And the most common symptom was anemia(20 cases, 83.3%).The median hemoglobin level was 75(46-145) g/L.The median IgM level was 32.9(6.4-79.3) g/L.18 cases(75%) secreted kappa light chain and 6 cases(25%) secreted lambda light chain.In 16 patients with available immunophenotypic data, 13 cases(81.3%) expressed sIgM, CD19, CD20 and CD22, but missed the expression of CD5, CD10 and CD23.The median progression-free survival(PFS) was 7.5(1-51) months.Univariate analysis revealed that many factors influenced progression-free survival(PFS) of WM patients, including age, hemoglobin(Hb), platelet(PLT), β2 microglobulin(β2-MG), IgM concentration, albumen(ALB), serum creatinine(SCr), lactate dehydrogenase(LDH), C-reactive protein(CRP) and severe immune paralysis.Patients with the application of chemotherapy regimens containing rituximab or bortezomib had longer PFS than those who did not apply.Based on Cox multivariate analysis, the independent adverse prognostic factors include age(P=0.008),IgM concentration(P=0.028) and SCr(P=0.005).CONCLUSIONS WM often occurs in elderly men and secretes kappa light chain.The clinical course of WM is indolent B-cell lymphoma.Age, IgM concentration and SCr are independent adverse prognostic factors.The application of rituximab or bortezomib is expected to extend PFS of WM.