Liu Wenlin, Zhou Yue, Li Lingyan, Zhang Fan, Xiong Zuying, Hou Shuang
Department of Nephrology, Peking University Shenzhen Hospital, Shenzhen, Guangdong Province, 518000, China.
BMC Nephrol. 2024 Dec 24;25(1):472. doi: 10.1186/s12882-024-03898-z.
POEMS syndrome with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes is an uncommon plasma cell paraneoplastic syndrome involving multiple system. It is relatively rare in clinical practice, and renal involvement is a usual yet easily overlooked symptom.
We successfully treated a patient with M protein-negative POEMS syndrome with membranoproliferative glomerulonephritis (MPGN) findings and thrombotic microangiopathic changes by comparing the level of Vascular endothelial growth factor (VEGF) in the serum and the changes in polyserositis before and after the patient's treatment.
POEMS syndrome clinically involves multiple systems and has complex symptoms. Because of the diversity of the disease manifestations, identification of atypical POEMS syndrome and timely intervention are important for patient survival and prognosis.
POEMS综合征伴有多发性神经病、器官肿大、内分泌病、M蛋白和皮肤改变,是一种累及多系统的罕见浆细胞副肿瘤综合征。在临床实践中相对少见,肾脏受累是常见但易被忽视的症状。
通过比较患者治疗前后血清中血管内皮生长因子(VEGF)水平及多浆膜炎的变化,我们成功治疗了一名患有膜增生性肾小球肾炎(MPGN)表现和血栓性微血管病变的M蛋白阴性POEMS综合征患者。
POEMS综合征临床累及多个系统且症状复杂。由于疾病表现的多样性,识别非典型POEMS综合征并及时干预对患者的生存和预后很重要。