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IgD浆细胞瘤:临床表现及特征

IgD plasma cell neoplasia: clinical manifestations and characteristic features.

作者信息

Pruzanski W, Rother I

出版信息

Can Med Assoc J. 1970 May 23;102(10):1061-5.

Abstract

Three patients, one with plasma cell leukemia and clinically asymptomatic hypernephroma and meningioma, and two others with multiple myeloma, had M-components of IgD/lambda type. In the first case, IgD globulin was found in the serum, ascitic and pleural fluids. Including our patients, 50 cases of IgD myeloma have been reported in the literature. A review of this group showed some significant differences from the other classes of multiple myeloma. IgD myeloma seems to involve a larger proportion of younger people, 66% being less than 59 years of age. The involvement of internal organs and renal damage were more frequent in IgD myeloma than in other classes. Serum total protein was frequently not increased, the relative concentration of M-component was often low and in 12% there was no spike in electrophoresis. The diagnosis therefore was sometimes difficult. In a quarter of the cases Bence Jones proteinemia was found and in 15% there were multiple spikes, both these manifestations being rare in IgG or IgA classes of myeloma. In 89%, IgD globulin had lambda type light chain, clearly contrasting with the figure of approximately 30% in other classes. Bence Jones protein was found in the urine in 91%. The survival time seemed to be shorter than in other myelomas.

摘要

三名患者,一名患有浆细胞白血病、临床无症状性肾细胞癌和脑膜瘤,另外两名患有多发性骨髓瘤,其M成分均为IgD/λ型。在第一例中,血清、腹水和胸水中均发现了IgD球蛋白。包括我们的患者在内,文献中已报道了50例IgD骨髓瘤。对该组病例的回顾显示,其与其他类型的多发性骨髓瘤存在一些显著差异。IgD骨髓瘤似乎在年轻人中所占比例更大,66%的患者年龄小于59岁。与其他类型相比,IgD骨髓瘤累及内脏器官和造成肾损害更为常见。血清总蛋白常常不升高,M成分的相对浓度往往较低,12%的患者在电泳中无峰。因此,诊断有时很困难。四分之一的病例发现有本周氏蛋白尿,15%的病例有多个峰,这两种表现在IgG或IgA类骨髓瘤中均很少见。89%的患者IgD球蛋白具有λ型轻链,这与其他类型中约30%的比例形成明显对比。91%的患者尿液中发现了本周氏蛋白。其生存时间似乎比其他骨髓瘤患者更短。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3f61/1930256/871dacc495fe/canmedaj01593-0049-a.jpg

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