POEMS综合征伴病理性骨折一例  

A case of POEMS syndrome with pathological fracture

作　　者：狄鹤轩 齐典文[1] 赵茗[1] 张国川[1] DI He-xuan;QI Dian-wen;ZHAO Ming;ZHANG Guo-chuan(Bone and Soft Tissue Oncology,Hebei Medical University Third Hospital,Shijiazhuang,Hebei,050051,China)

机构地区：[1]河北医科大学第三医院骨与软组织肿瘤科,石家庄050051

出　　处：《中国骨与关节杂志》2024年第9期749-752,共4页Chinese Journal of Bone and Joint

摘　　要：POEMS综合征是一种多系统受累的浆细胞疾病,临床表现包括多发性神经疾病(polyneuropathy)、脏器肿大(organomegaly)、内分泌疾病(endocrinopathy)、M蛋白(M-protein)、皮肤改变(skin changes),POEMS综合征是由Bardwick等[1]学者根据5个临床表现的首字母提出的名称,少数POEMS综合征的患者存在溶骨性病变[2]。这篇文章的目的在于提示POEMS综合征溶骨性骨病变可发生病理性骨折,应用3D打印技术治疗取得了良好疗效,现报道如下并行文献复习。Objective To describe the surgical treatment of a case of POEMS syndrome with a pathologic fracture of the femur and review of the relevant literature.Methods The surgical resection and application of 3D printed prosthesis reconstruction to treat a case of POEMS syndrome combined with pathologic fracture were described.The diagnosis of POEMS syndrome with pathologic fracture was made through a series of tests and laboratory tests,such as blood routine,electromyography,pulmonary function test,and surgical pathology.A 3D-printed prosthesis with preserved knee joint was designed for the patient before surgery.The pain visual analogue scale was used to assess the surgical outcome,and the MSTS 93 scale was used to assess the clinical efficacy at the final follow-up.Results Surgery completely resected the right femoral lesion and reconstructed limb function.The patient’s VAS score decreased from 8 preoperatively to 3 postoperatively.One year postoperatively,the patient’s surgical incision was well healed and the prosthesis was in position with no deformity of the hip.At the final followup,the patient’s MSTS 93 score was 22.An individualized rehabilitation plan was developed for the patient in order to achieve better functional recovery.Conclusions Surgical resection and reconstruction are effective for patients with POEMS syndrome with pathologic fractures.3D-printed customized prosthesis is a promising reconstruction technique for patients with long segmental lesions.Although there is no standardized treatment for POEMS syndrome with pathologic fracture,the prognosis and quality of life can be improved through active comprehensive treatment.

关 键 词：浆细胞瘤 股骨 POEMS综合征 骨肿瘤 

分 类 号：R687.4[医药卫生—骨科学]