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伴有混合性冷球蛋白血症的免疫母细胞性淋巴结病。一项详细病例研究。

Immunoblastic lymphadenopathy with mixed cryoglobulinemia. A detailed case study.

作者信息

Schultz D R, Yunis A A

出版信息

N Engl J Med. 1975 Jan 2;292(1):8-12. doi: 10.1056/NEJM197501022920102.

Abstract

The premise that chronic antigenic stimulation may be involved in lymphoproliferative disorders was considered in a patient with immunoblastic lymphadenopathy who had received liver extract by injection and by mouth for many years. The salient features were lymphadenopathy and hepatosplenomegaly, a predominance of lymphocytes and plasmacytoid cells with mitotic figures in lymph-node imprints, a cryoglobulin containing IgG, IgA, IgM and bound complement components, depressed serum complement levels, and Coombs-test-positive erythrocytes. Immunoglobulin concentrations per 100 ml of serum were IgG, 5900 mg, IgA, 1480 mg, and IgM, 5640 mg, with normal ranges of 710 to 1540, 60 to 490, and 37 to 204 mg, respectively. Serum precipitins to an antigen (or antigens) in the liver extract resided in the IgA and IgM classes. Complete remission followed one course of cyclophosphamide, vincristine, and prednisone. We propose that the syndrome was caused by chronic antigenic stimulation with liver extract.

摘要

一位患有免疫母细胞性淋巴结病的患者多年来一直通过注射和口服方式接受肝提取物治疗,人们由此考虑慢性抗原刺激可能与淋巴增生性疾病有关。该患者的显著特征包括淋巴结病和肝脾肿大,淋巴结印片中淋巴细胞和浆细胞样细胞占优势且有丝分裂象,存在一种含有IgG、IgA、IgM和结合补体成分的冷球蛋白,血清补体水平降低,以及抗人球蛋白试验阳性红细胞。每100 ml血清中的免疫球蛋白浓度分别为:IgG 5900 mg、IgA 1480 mg、IgM 5640 mg,正常范围分别为710至1540 mg、60至490 mg、37至204 mg。针对肝提取物中一种(或多种)抗原的血清沉淀素存在于IgA和IgM类别中。经过一个疗程的环磷酰胺、长春新碱和泼尼松治疗后完全缓解。我们认为该综合征是由肝提取物的慢性抗原刺激引起的。

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