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一例μ重链病:μ链病与巨球蛋白血症的合并特征。

A case of mu-heavy chain disease: combined features of mu-chain disease and macroglobulinemia.

作者信息

Iwasaki T, Hamano T, Kobayashi K, Kakishita E

机构信息

Second Department of Internal Medicine, Hyogo College of Medicine, Japan.

出版信息

Int J Hematol. 1997 Oct;66(3):359-65. doi: 10.1016/s0925-5710(97)00039-x.

DOI:10.1016/s0925-5710(97)00039-x
PMID:9401282
Abstract

A case of mu-heavy chain disease (HCD) is described. The patient, a 40-year-old man, presented with an intracranial tumor. The bone marrow of this patient showed infiltration with both plasma cells and lymphocytes. The majority of plasma cells were vacuolated and the result of immunoelectrophoresis of serum protein revealed an arc with anti-IgM antiserum and an additional rapid migrating arc of different mobility with anti-kappa antiserum. The urine contained a kappa-type Bence Jones protein. An enzyme-linked antibody study showed that the majority of plasma cells in bone marrow contained both mu and kappa antigenic determinants in their cytoplasm. On Sephadex G-200 gel filtration, the monoclonal IgM-kappa protein and the mu-chain fragment were detected in the serum, suggesting the combined features of mu-HCD and macroglobulinemia. The molecular weight of the mu-chain fragment was approximately 45,000 daltons. The intracranial tumor completely disappeared after irradiation therapy. However, he died 1 year later after development of a huge abdominal tumor.

摘要

报告1例μ重链病(HCD)。患者为40岁男性,表现为颅内肿瘤。该患者骨髓显示浆细胞和淋巴细胞浸润。大多数浆细胞呈空泡状,血清蛋白免疫电泳结果显示,抗IgM抗血清出现一条弧,抗κ抗血清出现一条迁移速度不同的额外快速迁移弧。尿液中含有κ型本-周蛋白。酶联抗体研究显示,骨髓中大多数浆细胞在其细胞质中同时含有μ和κ抗原决定簇。在Sephadex G-200凝胶过滤中,血清中检测到单克隆IgM-κ蛋白和μ链片段,提示μ-HCD和巨球蛋白血症的联合特征。μ链片段的分子量约为45,000道尔顿。颅内肿瘤经放射治疗后完全消失。然而,1年后他因出现巨大腹部肿瘤而死亡。

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