POMES综合征1例报道并临床分析

POMES Syndrome:A Case Report and Clinical Analysis

目的报告1例以神经系统病变为首发症状,合并脏器肿大、内分泌系统功能紊乱及骨质增生的POMES综合征病例。方法回顾性分析POMES综合征的临床表现、骨髓活检结果、诊疗经过,结合文献对POMES综合征临床特点、诊断标准及治疗进行探讨。结果老年男性患者,临床表现为四肢无力伴麻木感、疼痛2个月余,尿轻链测定显示κ,λ链异常,B超检查示脏器肿大,肌电图检查示神经源性损害,PET-CT检查示有不同程度的骨质增生,骨髓涂片显示粒系、红系增生活跃。结论对于出现神经系统症状,合并脏器肿大、内分泌系统功能紊乱及骨质增生的患者应考虑POMES综合征可能。

Aim One patient who had POMES syndrome with neuropathy as an initial symptom associated with organomegaly, endocrinopathy and hyperosteogeny was described. Methods A case diagnosed as POMES syndrome was reported and the characteristics of POMES syndrome were reviewed. Results The patient developed limbs weakness and numbness for more than two months. Urinary light chain assay showed abnormal κ,λ chain, B ultrasonic displayed organomegaly, EMG showed neurogenic damage, PET-CT showed varying degrees of bone hyperplasia, bone marrow smears showed active hyperplasia of myeloid and erythroid. Conclusion For patients who had neurological symptoms, arganomegaly endocrinopathy and hyperosteogeny, POMES sydrome should be taken into consideration.