原发性巨球蛋白血症临床甚为罕见,骨髓及组织中淋巴样浆细胞增生,常与异常球蛋白M 蛋白增高有密切关系;肺弥漫性间质性纤维化又称 Hamman—Rich 二氏综合征,亦较少见,近年认为是一种自身免疫性疾病。最近我院遇到原发性巨球蛋白血症并发慢性肺弥漫性纤维化一例,鉴于本病少见、发病机理尚欠清楚,诊断与治疗均有待改进,特提出报告,以供参考。 Primary macroglobulinemia clinical rare, lymphoid plasma cells in bone marrow and tissue hyperplasia, often associated with abnormal globulin M protein is closely related to; pulmonary interstitial fibrosis, also known as Hamman-Rich’s Syndrome, is also less common in recent years that is an autoimmune disease. Recently, our hospital met with a case of primary macroglobulinemia complicated by chronic pulmonary fibrosis, in view of the rare disease, the pathogenesis is not clear, diagnosis and treatment have yet to be improved, special report for reference.