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一名患有脱髓鞘性多发性神经病及POEMS综合征异常变体患者的“良性”肋骨病变的自然病史。

The natural history of a "benign" rib lesion in a patient with a demyelinating polyneuropathy and an unusual variant of POEMS syndrome.

作者信息

Simmons Z, Wald J, Albers J W, Feldman E L

机构信息

Division of Neurology, Pennsylvania State University College of Medicine, Milton S. Hershey Medical Center, Hershey 17033.

出版信息

Muscle Nerve. 1994 Sep;17(9):1055-9. doi: 10.1002/mus.880170913.

Abstract

Patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome exhibit great clinical variability and often have osteosclerotic myeloma. We present a patient with an acquired demyelinating polyneuropathy who was eventually diagnosed as having POEMS syndrome. Her long period of observation permitted documentation of the natural history of a plasmacytoma, including its remarkably slow rate of growth and its transformation from a nonsclerotic to a sclerotic bone lesion. Her clinical and laboratory features emphasize the variability of this syndrome. Biopsy of benign-appearing bone lesions should be considered in patients with acquired demyelinating polyneuropathies who do not respond to standard treatment modalities. Serial x-rays may not distinguish benign lesions from plasmacytomas in this treatable condition.

摘要

POEMS(多发性神经病、器官肿大、内分泌病、M蛋白和皮肤改变)综合征患者临床表现差异很大,常伴有骨硬化性骨髓瘤。我们报告一例获得性脱髓鞘性多发性神经病患者,最终被诊断为POEMS综合征。对其进行的长期观察记录了浆细胞瘤的自然病程,包括其显著缓慢的生长速度以及从非硬化性骨病变转变为硬化性骨病变的过程。她的临床和实验室特征突出了该综合征的变异性。对于对标准治疗方式无反应的获得性脱髓鞘性多发性神经病患者,应考虑对看似良性的骨病变进行活检。在这种可治疗的情况下,连续X线检查可能无法区分良性病变和浆细胞瘤。

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