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浆细胞异常增生症、多发性神经病和糖尿病综合征。

Syndrome of plasma cell dyscrasia, polyneuropathy, and diabetes mellitus.

作者信息

Davies M L, Elson D L, Hertz D, McMillin J M

机构信息

Department of Medicine, University of South Dakota School of Medicine, Sioux Falls.

出版信息

West J Med. 1990 Mar;152(3):257-60.

Abstract

Multiple myeloma associated with sclerotic bone lesions and polyneuropathy represents a distinct subset of the plasma cell dyscrasias. We describe a case of biclonal gammopathy (the second case reported), insulin-resistant diabetes mellitus, and no evidence for anti-insulin receptor antibodies. After treatment with chemotherapy and irradiation, the diabetes resolved, the polyneuropathy lessened greatly, and the patient is alive without evidence of progression five years later. The reports of 95 other cases are reviewed. This syndrome occurs in younger patients (mean age, 48 years) and is frequently associated with organomegaly, endocrinopathies, and skin changes. Irradiation to the sclerotic bone lesions frequently lessens the neuropathy and endocrinopathies and may result in long-term remission. The mechanism of action leading to the systemic effects seen in this syndrome is unknown but is likely related to proteins secreted by the abnormal plasma cells.

摘要

伴有硬化性骨病变和多发性神经病的多发性骨髓瘤是浆细胞发育异常中的一个独特亚型。我们描述了一例双克隆丙种球蛋白病(第二例报道)、胰岛素抵抗型糖尿病且无抗胰岛素受体抗体证据的病例。经过化疗和放疗后,糖尿病得到缓解,多发性神经病大为减轻,患者五年后仍存活且无病情进展迹象。我们还回顾了其他95例病例的报告。该综合征发生于较年轻患者(平均年龄48岁),常伴有器官肿大、内分泌病和皮肤改变。对硬化性骨病变进行放疗常常可减轻神经病和内分泌病,并可能导致长期缓解。导致该综合征中所见全身效应的作用机制尚不清楚,但可能与异常浆细胞分泌的蛋白质有关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e00a/1002325/a9844cf84032/westjmed00115-0044-a.jpg

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