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Individualizing treatment for Waldenstrom's macroglobulinemia

机译:Waldenstrom巨球蛋白血症的个体化治疗

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Waldenstrom's macroglobulinemia (WM) is a distinct B-cell lymphoprolifer-ative disorder primarily characterized by lymphoplasmacytic cells infiltrating the bone marrow (BM), along with demonstration of an IgM monoclonal gammo-pathy [i-4]. According to the Revised European American Lymphoma (REAL) and WHO systems, WM is classified as a lymphoplasmacytic lymphoma [3,4]. Each year in the USA, there are 1500 new cases diagnosed and the median age of diagnosis is 60 years; the overall incidence of WM is approximately three per million persons per year [5-7]. In 11 population-based registries within the USA, the age-adjusted rates for men and women are 3.4 and 1.7 per million, respectively. Rates increased sharply with age: 0.1 per million individuals under 45 years of age and 36.3 per million individuals over 75 years of age. These rates are comparable to those for hairy-cell leukemia, and do not appear to be increasing. In addition, the rates are clearly higher for Caucasians than for African-Americans [5,8]. The median overall survival of patients with WM is 5?years; however, a recent study of 337 patients with symptomatic WM showed a median disease-specific survival of 11.2 years [7].
机译:Waldenstrom的巨球蛋白血症(WM)是一种独特的B细胞淋巴组织增生性疾病,其主要特征是浸润骨髓(BM)的淋巴浆细胞,以及IgM单克隆配子病[i-4]的表现。根据修订的欧美淋巴瘤(REAL)和WHO系统,WM被归类为淋巴浆细胞性淋巴瘤[3,4]。在美国,每年诊断出1500例新病例,诊断的平均年龄为60岁。每年,WM的总发病率约为百万分之三[5-7]。在美国的11个以人口为基础的注册中心中,按年龄调整的男性和女性比率分别为百万分之3.4和1.7。发病率随着年龄的增长而急剧上升:45岁以下的人口中每百万人中有0.1人,75岁以上的人口中每百万中有36.3人。这些比率与毛细胞白血病的比率相当,并且似乎没有增加。此外,高加索人的比率明显高于非裔美国人[5,8]。 WM患者的平均总生存期为5年。然而,最近对337例有症状WM患者的研究显示,疾病特异性生存中位数为11.2年[7]。

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