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[POEMS综合征的一个例证病例]

[An illustrative case of the POEMS syndrome].

作者信息

Radermecker A, Bonnet C, Lutteri L, Chapelle A C, Petignot S, Lievens I, Caers J

机构信息

Service d'Ophtalmologie, CHU Liège, Belgique.

Service d'Hématologie, CHU Liège, Belgique.

出版信息

Rev Med Liege. 2021 Mar;76(3):156-159.

Abstract

POEMS syndrome is a rare and invalidating entity characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and dermatoses. The diagnosis of this condition is often late and challenging due to the heterogeneity of clinical forms. The light chains secreted by the clonal plasmocytes cause overproduction of VEGF (Vascular Endothelial Growth Factor) responsible for the appearance of the clinical manifestations of POEMS. The diagnostic approach is based on different clinical and biological criteria. Patients with a solitary plasmacytoma are candidates for radiotherapy treatment. Patients with diffuse bone involvement or bone marrow infiltration are best treated by systemic drugs. The response to treatment may take several months before clinical and biological improvement. Early diagnosis and dedicated management limit the clinico-functional impact of POEMS.

摘要

POEMS综合征是一种罕见且使人衰弱的病症,其特征为多发性神经病、器官肿大、内分泌病、单克隆丙种球蛋白病和皮肤病。由于临床症状的异质性,这种病症的诊断往往较晚且具有挑战性。克隆性浆细胞分泌的轻链导致血管内皮生长因子(VEGF)过度产生,从而引发POEMS的临床表现。诊断方法基于不同的临床和生物学标准。孤立性浆细胞瘤患者适合接受放射治疗。弥漫性骨受累或骨髓浸润的患者最好采用全身药物治疗。治疗反应可能需要数月时间才能实现临床和生物学改善。早期诊断和专门管理可限制POEMS的临床功能影响。

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