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
DescriptionA patient with history of neck swelling 患者颈部肿胀
LegendsContrast-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 PresentationSymmetrical 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
RxMultisystem 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
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