华氏巨球蛋白血症的临床研究
发布时间:2018-09-07 15:02
【摘要】:目的总结华氏巨球蛋白血症(Waldenstr(?)m's macroglobulinemia,WM)的临床特点和诊治情况,分析影响预后的相关因素。方法回顾性分析2011年1月至2016年1月于郑州大学人民医院诊治的24例WM患者的临床资料。结果24例WM患者,男女比例2:1(男16例,女8例),中位年龄62.5(42-79)岁;最常见的临床症状是疲乏(20例,83.3%);中位血红蛋白(Hb)水平75(46~145)g/L;中位IgM水平32.9(6.4~79.3)g/L;IgMκ型18例(75%),IgMλ型6例(25%)。8例患者行骨髓活检,其中3例网状纤维染色为(++~+++)。16例行流式细胞术检测,其中13例(81.3%)表现为sIgM+CD19+CD20+CD22+CD5-CD10-CD23-。全组中位无进展生存时间(PFS)7.5(1-51)个月。单因素分析结果示,年龄、Hb、血小板、IgM水平、白蛋白、β2微球蛋白、C反应蛋白、乳酸脱氢酶、血清肌酐(SCr)及合并重度免疫不全麻痹可影响患者PFS,应用含利妥昔单抗或硼替佐米化疗方案组的PFS相对较长。多因素分析结果示,年龄(P=0.008)、IgM水平(P=0.028)及SCr(P=0.005)是WM预后不良的独立危险因素。根据年龄、IgM水平和SCr可将WM患者分为危险程度不同的三组,三组中位PFS分别是26个月、6个月、2个月(P0.001)。结论WM好发于老年男性,以IgMκ型多见,临床表现多样且无特异性,国内WM患者发病年龄较小,且临床表现较重。WM具有惰性B细胞淋巴瘤的特点,可伴骨髓纤维化。根据年龄、IgM水平和SCr对WM患者分组有利于预后评估,利妥昔单抗或硼替佐米的应用有望提高疗效并改善预后。
[Abstract]:Objective to summarize the clinical features, diagnosis and treatment of (Waldenstr (? M macroglobulinemia,WM and to analyze the related prognostic factors. Methods the clinical data of 24 patients with WM from January 2011 to January 2016 in Renmin Hospital of Zhengzhou University were retrospectively analyzed. Results Twenty-four patients with WM were diagnosed at 2:1 (16 males and 8 females) with median age of 62.5 (42-79). The most common clinical symptoms were fatigue (20 cases, 83.3%), median hemoglobin (Hb) level (75 (460.145) g / L), median IgM level (32.9 (6.479.3) g / L IgM 魏 type (18 cases, 75%), bone marrow biopsy in 6 cases (25.8%). Among them, 3 cases were stained with (~) .16 cases were detected by flow cytometry, 13 cases (81.3%) showed sIgM CD19 CD20 CD22 CD5-CD10-CD23-.. The median progression-free survival time (PFS) was 7.5 (1-51) months. Univariate analysis showed that the age of HB, platelet titer IgM, albumin, 尾 2 microglobulin C reactive protein, lactate dehydrogenase, Serum creatinine (SCr) and severe immune insufficiency could affect the PFS of patients with PFS, treated with Rituximab or bortezomil chemotherapy regimen. Multivariate analysis showed that age (P0. 008) and SCr (P0. 005) were independent risk factors for poor prognosis of WM. The patients with WM were divided into three groups according to age and SCr. The median PFS of the three groups was 26 months, 6 months and 2 months (P0.001). Conclusion WM is more common in the elderly men, and its clinical manifestations are diverse and non-specific. The patients with WM in China have the characteristics of inert B-cell lymphoma and their clinical manifestations are characterized by inert B-cell lymphoma, which can be accompanied by bone marrow fibrosis. The classification of WM patients according to age level and SCr is beneficial to the prognosis evaluation. The application of rituximab or bortezomil is expected to improve the curative effect and improve the prognosis.
【学位授予单位】:郑州大学
【学位级别】:硕士
【学位授予年份】:2017
【分类号】:R733
,
本文编号:2228602
[Abstract]:Objective to summarize the clinical features, diagnosis and treatment of (Waldenstr (? M macroglobulinemia,WM and to analyze the related prognostic factors. Methods the clinical data of 24 patients with WM from January 2011 to January 2016 in Renmin Hospital of Zhengzhou University were retrospectively analyzed. Results Twenty-four patients with WM were diagnosed at 2:1 (16 males and 8 females) with median age of 62.5 (42-79). The most common clinical symptoms were fatigue (20 cases, 83.3%), median hemoglobin (Hb) level (75 (460.145) g / L), median IgM level (32.9 (6.479.3) g / L IgM 魏 type (18 cases, 75%), bone marrow biopsy in 6 cases (25.8%). Among them, 3 cases were stained with (~) .16 cases were detected by flow cytometry, 13 cases (81.3%) showed sIgM CD19 CD20 CD22 CD5-CD10-CD23-.. The median progression-free survival time (PFS) was 7.5 (1-51) months. Univariate analysis showed that the age of HB, platelet titer IgM, albumin, 尾 2 microglobulin C reactive protein, lactate dehydrogenase, Serum creatinine (SCr) and severe immune insufficiency could affect the PFS of patients with PFS, treated with Rituximab or bortezomil chemotherapy regimen. Multivariate analysis showed that age (P0. 008) and SCr (P0. 005) were independent risk factors for poor prognosis of WM. The patients with WM were divided into three groups according to age and SCr. The median PFS of the three groups was 26 months, 6 months and 2 months (P0.001). Conclusion WM is more common in the elderly men, and its clinical manifestations are diverse and non-specific. The patients with WM in China have the characteristics of inert B-cell lymphoma and their clinical manifestations are characterized by inert B-cell lymphoma, which can be accompanied by bone marrow fibrosis. The classification of WM patients according to age level and SCr is beneficial to the prognosis evaluation. The application of rituximab or bortezomil is expected to improve the curative effect and improve the prognosis.
【学位授予单位】:郑州大学
【学位级别】:硕士
【学位授予年份】:2017
【分类号】:R733
,
本文编号:2228602
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