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免疫母细胞性淋巴结病患者中具有干燥综合征和系统性红斑狼疮特征的免疫母细胞肉瘤

Immunoblastic sarcoma with features of Sjögren's syndrome and systemic lupus erythematosus in a patient with immunoblastic lymphadenopathy.

作者信息

Pierce D A, Stern R, Jaffe R, Zulman J, Talal N

出版信息

Arthritis Rheum. 1979 Aug;22(8):911-6. doi: 10.1002/art.1780220816.

Abstract

A patient with immunoblastic lymphadenopathy and features of Sjögren's syndrome and systemic lupus erythematosus is presented. Clinical features included generalized lymphadenopathy, rash, alopecia, and synovitis, with associated laboratory abnormalities of a positive antinuclear factor and double-stranded DNA antibodies, positive lupus band test, and hemolytic anemia. Symptoms of Sjögren's syndrome included dry eyes and mouth and swollen parotid gland; biopsy results of the accessory salivary glands were positive. At autopsy immunoblastic sarcoma was found that involved the myocardium, which stained for both kappa and lambda light chains by immunoperoxidase techniques.

摘要

本文报告了一例患有免疫母细胞性淋巴结病,并伴有干燥综合征和系统性红斑狼疮特征的患者。临床特征包括全身淋巴结肿大、皮疹、脱发和滑膜炎,实验室检查异常包括抗核因子和双链DNA抗体阳性、狼疮带试验阳性以及溶血性贫血。干燥综合征的症状包括眼干、口干和腮腺肿大;副唾液腺活检结果为阳性。尸检发现免疫母细胞肉瘤累及心肌,通过免疫过氧化物酶技术检测,该肿瘤对κ和λ轻链均呈阳性染色。

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