Gallart M T, Canals J, Cañadell E, Cortez L, Moragas A, Schwartz S
Acta Haematol. 1978;59(5):262-76. doi: 10.1159/000207781.
The first case of gamma-heavy chain disease described in Spain is here reported. The patient, a 36-year-old woman, presented fever, enlarged regional lymph nodes, and hepatosplenomegaly, without bone marrow abnormalities but with lymphopenia. Serum electrophoresis did not disclose any M-component. The abnormal gamma-chain protein had a alpha2-globulin mobility and was immunochemically related to the Fc fragment. It belonged to the IgG 4 subclass, its molecular weight was about 60,000. Proteinuria was minimal but the electrophoresis of concentrated urine showed a homogeneous peak of alpha2-globulin mobility constituted by the gamma-chain fragment. Biopsy of an axillary lymph node disclosed features of immunoblastic sarcoma. The course was malignant, resulting in death in 8 months.
本文报告了西班牙首例γ重链病病例。患者为一名36岁女性，有发热、局部淋巴结肿大和肝脾肿大症状，骨髓无异常但有淋巴细胞减少。血清电泳未发现任何M成分。异常的γ链蛋白具有α2球蛋白迁移率，且在免疫化学上与Fc片段相关。它属于IgG 4亚类，分子量约为60,000。蛋白尿轻微，但浓缩尿电泳显示由γ链片段构成的α2球蛋白迁移率的均匀峰。腋窝淋巴结活检显示免疫母细胞肉瘤特征。病程呈恶性，8个月后死亡。
