POEMS综合征

来自：双语学影像 ；本期病例选自AJNR Case of the Week

Stephen Price; Wilson Altmeyer, MD；Department of Radiology；University of Texas Health Science Center at San Antonio；San Antonio, TX

Description

A patient with history of neck swelling

患者颈部肿胀

Legends

Contrast-enhanced CT scan, coronal plane (A) demonstrates cervical lymphadenopathy. Sagittal reformatted CT scan, bone window (B) demonstrates numerous sclerotic foci in the manubrium sternum and the thoracic vertebrae. Coronal CT scan (C) demonstrates hepatomegaly.

增强CT冠状位重建显示颈部淋巴结病。

CT矢状位重建示胸骨柄及胸椎椎体多发局灶性硬化。

CT冠状位示肝脏肿大。

POEMS Syndrome

POEMS综合征

POEMS syndrome is a poorly understood entity characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein (monoclonal plasma cell proliferative disorder), and skin changes.

POEMS综合征既往很少被了解，其本质特点为多发神经病变、脏器增大、内分泌病变、M-蛋白（单克隆浆细胞增殖性病变）、及皮肤改变。

Etiology not established, likely secondary to imbalance of inflammatory cytokines.

POEMS综合征的病因尚不明确，可能继发于炎性细胞因子失衡。

Clinical Presentation

Symmetrical and ascending peripheral neuropathy most common manifestation, pulmonary manifestations (PAH, restrictive lung disease), endocrinopathy (most commonly gonadal axis). Skin changes include scleroderma, hyperpigmentation, hemangioma/telangiectasia.

对称性上行周围神经病变最多见、肺部表现（肺动脉高压、限制性肺疾病），内分泌疾病（最常见于生殖腺轴）、皮肤改变包括硬皮病、色素沉着、血管瘤/毛细血管扩张。

Key Diagnostic Features

• Radiographic findings of POEMS syndrome are nonspecific.

  POEMS综合征的影像学表现缺乏特异性。
  

• Almost always associated with sclerotic bone lesions

  大部分合并骨骼硬化病变

• Organomegaly(hepatomegaly, splenomegaly) may be noted.

  常可见脏器增大（肝肿大、脾大）

• Lymphadenopathy(including Castleman's disease) is a common feature.

  常见淋巴结病（包括Castleman病）

• Lymphoma

  淋巴瘤

• Osteoblasticmetastatic disease

  成骨性转移瘤

• Multiplemyeloma

  多发性骨髓瘤

Rx

• Multisystem disease, no standardized therapy.

  对于多系统病变，目前尚无明确的治疗标准

• Radiation may be utilized in a solitary bone lesion.

  放疗对孤立性骨骼病变可能有效

• High-dose chemotherapy with autologous stem cell support

  高剂量化疗及自身肝细胞支持