本文選題：華氏巨球蛋白血癥 + 淋巴瘤�。� 參考：《中華腫瘤防治雜志》2017年08期

【摘要】：目的華氏巨球蛋白血癥(Waldenstr9m’s macroglobulinemia,WM)是一種特殊類型的非霍奇金淋巴瘤,其發(fā)病率極低,國內外相關數(shù)據(jù)較為缺乏。本研究旨在探討WM的臨床特點、預后因素及診治方法。方法回顧性分析2011-01-01-2016-01-01鄭州大學人民醫(yī)院診治的24例WM患者臨床資料。結果 24例WM患者,男16例,女8例,男女比例為2∶1,年齡42~79歲,中位年齡62.5歲;貧血(20例,83.3%)是最常見的臨床表現(xiàn),中位血紅蛋白水平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例患者行流式細胞術檢測,13例(81.3%)表現(xiàn)為sIgM~+CD5~-CD10~-CD19~+CD20~+CD22~+CD23~-。全組中位無進展生存時間(PFS)為7.5(1~51)個月。單因素分析結果顯示,年齡、血紅蛋白(Hb)、血小板(PLT)、β2微球蛋白(β2-MG)、IgM水平、白蛋白(ALB)、血清肌酐(SCr)、乳酸脫氫酶(LDH)、C反應蛋白(CRP)和合并重度免疫不全麻痹影響患者PFS,應用含有利妥昔單抗或硼替佐米的化療方案組PFS相對較長。多因素分析結果顯示,年齡(P=0.008)、IgM水平(P=0.028)和SCr(P=0.005)與預后相關。結論 WM好發(fā)于老年男性,以IgMκ型多見,具有惰性B細胞淋巴瘤的特點,年齡、IgM水平和SCr是影響WM預后的獨立危險因素,利妥昔單抗或硼替佐米的應用有望延長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.
【作者單位】： 鄭州大學人民醫(yī)院(河南省人民醫(yī)院)血液科;
【基金】：河南省2013年科技發(fā)展計劃(132102310184)
【分類號】：R733.1

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