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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。
Objective Waldenstr9m’s macroglobulinemia (WM) is a special type of non-Hodgkin’s lymphoma, its incidence is very low, the lack of relevant data at home and abroad. This study aimed to explore the clinical features of WM, prognostic factors and diagnosis and treatment methods. Methods The clinical data of 24 WM patients diagnosed and treated by People’s Hospital of Zhengzhou University from January 2011 to January 2016 were retrospectively analyzed. Results A total of 24 WM patients, 16 males and 8 females, had a male to female ratio of 2: 1, ranging in age from 42 to 79 years with a median age of 62.5 years. Anemia (20 cases, 83.3%) was the most common clinical manifestation. The hemoglobin level was 75 (46 ~ 145) g / L; the median IgM level was 32.9 (6.4 ~ 79.3) g / L, of which 18 were type kappa (75%) and 6 were lambda (25%). Thirteen patients (81.3%) showed sIgM ~ + CD5 ~ -CD10 ~ -CD19 ~ + CD20 ~ + CD22 ~ + CD23 ~ - in 16 patients undergoing flow cytometry. The median overall progression-free survival (PFS) was 7.5 (1 to 51) months. Univariate analysis showed that age, hemoglobin (Hb), platelet (PLT), β2-MG, IgM, albumin, serum creatinine, lactate dehydrogenase (LDH) , C-reactive protein (CRP), and severe parainfluency combined with severe immunocompromised pFS were associated with longer PFS in the chemotherapy regimen containing rituximab or bortezomib. Multivariate analysis showed that age (P = 0.008), IgM (P = 0.028) and SCr (P = 0.005) were associated with prognosis. CONCLUSIONS: WM is more common in elderly men and is more common in IgMK type. The characteristics of idiopathic B-cell lymphoma, age, IgM level and SCr are independent risk factors for prognosis of WM. The application of rituximab or bortezomib is expected Extend PFS.