Division of Hematology, Mayo Clinic, Rochester, MN.
Division of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, MN.
Mayo Clin Proc. 2018 Jun;93(6):739-746. doi: 10.1016/j.mayocp.2018.02.011. Epub 2018 Apr 12.
To determine the incidence of Waldenström macroglobulinemia (WM) in a strictly defined geographic area over a 50-year period.
All residents of Olmsted County with a diagnosis of WM, consisting of a monoclonal IgM protein of any size and/or 10% or more lymphoplasmacytic infiltration of the bone marrow along with anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, or hepatosplenomegaly requiring therapy, were identified from January 1, 1961, to December 31, 2010. Patients with smoldering WM, lymphoplasmacytic lymphoma with an IgG or IgA monoclonal protein, and those with an IgM monoclonal gammopathy of undetermined significance were excluded. The peripheral blood smears, bone marrow aspirates, and biopsy specimens were reviewed by an experienced hematopathologist.
Twenty-two patients were identified as having WM. The age-adjusted incidence rate for males was 0.92 per 100,000 person-years (95% CI, 0.44-1.39 per 100,000 person-years) and for females was 0.30 per 100,000 person-years (95% CI, 0.08-0.53 per 100,000 person-years) with an age- and sex-adjusted incidence of 0.57 per 100,000 person-years (95% CI, 0.33-0.81 per 100,000 person-years). When evaluated using a smoothing spline, there was no convincing evidence for a change in the incidence of WM over the past 50 years. Patients diagnosed with WM after 2000 had an approximately 2-fold excess mortality compared with the expected population mortality (standardized mortality ratio, 2.4; 95% CI, 0.64-6.0).
Waldenström macroglobulinemia is a rare malignancy, and the incidence in Olmsted County, Minnesota, has shown virtually no change over the past 50 years.
在一个严格定义的地理区域内，确定 50 年内瓦尔登斯特伦氏巨球蛋白血症 (WM) 的发病率。
从 1961 年 1 月 1 日至 2010 年 12 月 31 日，确定所有在明尼苏达州奥姆斯特德县被诊断为 WM 的居民，其诊断标准为：单克隆 IgM 蛋白，其大小为任何大小和/或骨髓中 10%或更多的淋巴浆细胞浸润，同时伴有贫血、全身症状、高粘滞血症、淋巴结病、或需要治疗的肝脾肿大。排除冒烟型 WM、有 IgG 或 IgA 单克隆蛋白的淋巴浆细胞淋巴瘤，以及有意义未定性的 IgM 单克隆丙种球蛋白血症的患者。外周血涂片、骨髓抽吸物和活检标本由经验丰富的血液病理学家进行审查。
共确定 22 例 WM 患者。男性的年龄调整发病率为 0.92/10 万人年（95%可信区间，0.44-1.39/10 万人年），女性为 0.30/10 万人年（95%可信区间，0.08-0.53/10 万人年），年龄和性别调整发病率为 0.57/10 万人年（95%可信区间，0.33-0.81/10 万人年）。使用平滑样条进行评估时，过去 50 年来 WM 的发病率没有明显变化的证据。2000 年后被诊断为 WM 的患者的死亡率与预期人口死亡率相比高出约 2 倍（标准化死亡率比，2.4；95%可信区间，0.64-6.0）。
瓦尔登斯特伦氏巨球蛋白血症是一种罕见的恶性肿瘤，明尼苏达州奥姆斯特德县的发病率在过去 50 年内几乎没有变化。
