POEMS综合征伴硬化型骨髓瘤的影像学表现

摘要

目的:探讨POEMS综合征伴硬化型骨髓瘤骨骼病变的影像学表现.方法:回顾性分析9例经临床及病理证实的POEMS综合征伴硬化型骨髓瘤患者的临床资料、实验室检查及影像学检查.重点分析其骨骼病变的X线、CT及MRI表现.结果:POEMS综合征伴硬化型骨髓瘤好发于男性,中位年龄50～60岁.主要临床表现为多发神经病变、脏器肿大、内分泌异常、M蛋白、皮肤改变、血管外容量负荷增加、硬化性骨病.实验室检查:单克隆浆细胞异常增殖(通常是λ型),血VEGF升高.骨损影像学表现:累及部位多为中轴骨,如脊椎、骨盆.骨损大小不一,最大径约7cm,最小径约1～2mm; 7例多发,2例单发;7例表现为骨质硬化型,3例表现为混合型(溶骨性骨质破坏伴周边硬化).结论:POEMS综合征是一种多脏器损害疾病,临床表现复杂,其伴发硬化型骨髓瘤的影像学表现有一定特征性,对早期诊断有一定帮助.%Purpose: To discuss the imaging features of bone lesions in POEMS syndrome with osteosclerotic myeloma. Methods: The clinical materials, laboratory results and imaging appearances of 9 POEMS syndrome patients with osteosclerotic myeloma confirmed by clinical and pathological results were retrospectively analyzed. The manifestations of bone lesions on X-ray, CT and MRI were analyzed. Results: Most cases of POEMS syndrome with osteosclerotic myeloma occurred in men, with an average age of 50-60 years old. The clinical features mainly included polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes, extravascular volume overload, and sclerotic bone lesions. Laboratory examination results of them were shown with monoclonal plasma cell-proliferative disorder(always λ),and elevated vascular endothelial growth factor.The imaging findings of bone lesions were that they often involved the axial skeleton, such as vertebrae and pelvis. Bone lesions were varied in size, with the maximum diameter about 7cm and the minimum about l-2mm. Seven cases were with multiple lesions, 2 case was with single lesion. Seven cases were shown with multiple bone sclerosis,3 cases with mixed appearances (osteolytic lesions with a sclerotic rim). Conclusion: POEMS syndrome is a multi-systemic disease with complicated manifestations, those with sclerotic myeloma are shown with some characteristic imaging features which could be helpful for the early diagnosis.