EPIDEMIOLOGIC & IMMUNOGENETIC STUDY OF MACROGLOBULINEMIA

瓦尔登斯特伦巨球蛋白血症（W.M.）是一种罕见而复杂的 淋巴组织增生性恶性肿瘤通常伴有明显的 淋巴增生性肿瘤、自身免疫性疾病和免疫性疾病 一级亲属的异常 的主要目的 拟采用病例对照和家系研究方法，通过病历检查 审查和采访W.M.和慢性 继发于感染，炎症过程， 自身免疫性疾病和慢性药物使用，并确定是否有 淋巴增生性恶性肿瘤和自身免疫性 W.M.一级亲属的疾病与一级病例相比 控制的亲属。 第二个目标是确定某些 HLA抗原类型在病例和对照之间的频率不同， 某些HLA抗原类型是否在W.M.患者中过量出现。 以及他们与W. M.的一级亲属其它淋巴增殖 恶性肿瘤或自身免疫性疾病的家庭有两个或两个以上的情况下， 这些疾病与对照组相比。 第三个目标是检查 免疫球蛋白谱和免疫电泳模式， 一级亲属，并将结果与流行病学和HLA相关 特色 最后，我们将比较WM的风险因素。与 慢性淋巴细胞性白血病，以确定是否流行病学相似性 组织病理学相似性 我们会确认所有的W. M.例 从1975年7月1日到12月31日，在巴尔的摩的十家医院被诊断出来， 1982. 对于病例对照研究，每个病例将单独匹配 (by年龄、性别、种族、医院和入院日期）与三个对照组： 一个患有除淋巴增生性恶性肿瘤以外的任何恶性肿瘤， 任何非恶性疾病，和慢性淋巴细胞白血病第三。 为家庭 我们将采访每一个活着的一级亲属，如果已经去世， 他们的近亲，并抽血进行HLA和免疫球蛋白研究， 每一个活着的一级亲属。 建议的综合、深入 流行病学和免疫遗传学研究是一种独特的方法， 增加对病因学和 Waldenstrom巨球蛋白血症的病理生理学特征。

Waldenstrom's macroglobulinemia (W.M.) is a rare and complex lymphoproliferative malignancy often associated with an apparent excess of lymphoproliferative neoplasms, autoimmune disorders, and immunologic abnormalities in first-degree relatives. The primary objective of the proposed case-control and family study is to examine by medical chart review and interview the postulated association between W.M. and chronic antigenic stimulation secondary to infections, inflammatory processes, autoimmune diseases and chronic drug use and to determine whether there is an increased proportion of lymphoproliferative malignancies and auto-immune diseases in first-degree relatives of W.M. cases compared with first-degree relatives of controls. A second objective is to determine whether certain HLA antigen types differ in frequency between cases and controls and whether certain HLA antigen types occur in excess among persons with W.M. and their first-degree relatives with W.M., other lymphoproliferative malignancies or autoimmune diseases in families with two or more cases of these diseases compared with controls. A third objective is to examine immunoglobulin profiles and immunoelectrophoresis patterns among cases and first-degree relatives and to correlate findings with epidemiologic and HLA characteristics. Finally we will compare risk factors for W.M. with those for chronic lymphocytic leukemia to determine if epidemiologic similarities parallel histopathologic similarities. We will identify all W.M. cases diagnosed at ten Baltimore hospitals from July 1, 1975 through December 31, 1982. For the case-control study each case will be individually matched (by age, sex, race, hospital and date of admission) with three controls: one with any malignancy other than a lymphoproliferative malignancy, one with any non-malignant disease, and a third with CLL. For the family studies we will interview each living first-degree relative or, if dead, their next-of-kin, and draw blood for HLA and immunoglobulin studies for each living first-degree relative. The proposed integrated, in-depth epidemiologic and immunogenetic study of W.M. is a unique approach to increase knowledge of the relationship between etiologic and pathophysiologic characteristics of Waldenstrom's macroglobulinemia.