24例华氏巨球蛋白血症临床分析

发布时间：2018-04-27 07:22

本文选题：华氏巨球蛋白血症 + 淋巴瘤　；参考：《中华肿瘤防治杂志》2017年08期

【摘要】：目的华氏巨球蛋白血症(Waldenstr9m’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IgMκ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。
[Abstract]:Objective Waldenstr9m macroglobulinemiaemia (WM) is a special type of non-Hodgkin's lymphoma with very low incidence and lack of relevant data at home and abroad. The purpose of this study was to investigate the clinical features, prognostic factors and diagnosis and treatment of WM. Methods the clinical data of 24 WM patients treated in Renmin Hospital of Zhengzhou University in 2011-01-01-2016-01-01 were analyzed retrospectively. Results 24 cases of WM, male 16 and female 8, the ratio of male to female was 2: 1, the age was 42 to 79 years old, the median age was 62.5 years old, the most common clinical manifestation was anaemia in 20 cases (83.3%). The median hemoglobin level was 79.3g / L, the median IgM level was 32.9 ~ 6.4g / L, in which there were 18 patients with 魏 type and 75 patients with kappa type, and 13 patients with lambda type were detected by flow cytometry (Siga ~ CD5-CD10m- CD19- CD20- CD22 ~ CD23-), and the results of flow cytometry were as follows: (1) Siga ~ CD5- CD10- CD20 ~ CD20 ~ CD22 ~ CD23-. The median progressive survival time (PFS) of the whole group was 7.5 / 1 / 51) months. The results of univariate analysis showed that age, HB, PLT, 尾 2 microglobulin (尾 2-MGG) IgM levels, Serum creatinine (Cr), lactate dehydrogenase (LDH), C-reactive protein (CRP) and severe immune insufficiency were associated with ALBX. The PFS was relatively long in patients treated with chemotherapy regimen containing favorable toxiximab or bortezomil. The multivariate analysis showed that the age of P0. 008 and SCrP0. 005) were correlated with the prognosis of P0. 028) and SCrP0. 005 (P 0. 028). Conclusion IgM 魏 type is the most common type of IgM 魏 type in aged males. Age and SCr levels are independent risk factors for the prognosis of WM. The application of rituximab or bortezomil is expected to prolong the prognosis of WM.
【作者单位】：郑州大学人民医院(河南省人民医院)血液科;
【基金】：河南省2013年科技发展计划(132102310184)
【分类号】：R733.1

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