EPIDEMIOLOGIC & IMMUNOGENETIC STUDY OF MACROGLOBULINEMIA

瓦尔登斯特罗姆巨球蛋白血症(W.M.)是一种罕见而复杂的 淋巴增生性恶性肿瘤通常与明显过量的 淋巴增生性肿瘤、自身免疫性疾病和免疫学 一级亲属的异常。该计划的主要目标是 建议病例对照和家族性研究采用病历检查 回顾和采访WM与慢性疾病之间的假设联系 继发于感染、炎症过程的抗原刺激， 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的比例增加 西医病例一级亲属与一级亲属患病情况比较 控制的亲属。第二个目标是确定某些 人类白细胞抗原类型在病例和对照组之间的频率不同， 某些人类白细胞抗原类型是否在W.M.患者中出现过多。 以及他们与W.M.和其他淋巴增生性疾病的一级亲属 有两个或两个以上病例的家庭中的恶性肿瘤或自身免疫性疾病 将这些疾病与对照组进行比较。第三个目标是检查 病例间免疫球蛋白谱和免疫电泳谱 并将研究结果与流行病学和人类白细胞抗原相关联。 特点。最后，我们将比较W.M.的风险因素与 以确定慢性淋巴细胞白血病的流行病学相似性 平行的组织病理学相似性。我们将确定所有的W.M.病例 从1975年7月1日到12月31日在巴尔的摩的十家医院确诊， 1982年。在病例对照研究中，每个病例将被单独配对 (按年龄、性别、种族、医院和入院日期)，有三个对照： 一人患有淋巴增生性恶性肿瘤以外的任何恶性肿瘤，一人 患有任何非恶性疾病，三分之一患有慢性淋巴细胞性白血病。为了这个家庭 我们将采访每一个在世的一级亲属，或者，如果死了， 他们的近亲，并抽取血液进行人类白细胞抗原和免疫球蛋白研究 每个人都是在世的一级亲属。建议的综合、深入的 W.M.的流行病学和免疫遗传学研究是一种独特的方法 增加对病因和疾病之间关系的认识 Waldenstrom巨球蛋白血症的病理生理特点。

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.