石小岩,
蔡薇,
马亮亮^,
大连医科大学附属第一医院 血液科，辽宁 大连 116011

详细信息 作者简介: 石小岩（1990-），女，主治医师。E-mail：861256206@qq.com



通讯作者: 马亮亮，教授。E-mail：maliangliangdy@163.com 中图分类号: R559


摘要:目的分析POEMS综合征患者的临床特征及诊治情况。方法回顾性分析2012年3月至2021年4月在大连医科大学附属第一医院诊治的10例POEMS综合征患者的临床资料，其中男性8例，女性2例，中位发病年龄57.5岁（39～77岁），发病至确诊中位时间17个月（4～48个月）。总结其临床特征、诊疗经过及转归。结果10例患者中，慢性起病9例，周围神经病变10例，器官肿大8例，皮肤改变8例，内分泌改变8例，血小板增多2例。9例患者初诊未明确诊断，初诊至确诊中位时间6个月（1～35个月）。9例接受了糖皮质激素单药或联合来那度胺、马法兰或硼替佐米等抗浆细胞治疗。因呼吸衰竭或疾病进展死亡3例，其余7例患者中，临床疗效评价7例达R_C；神经病变疗效评价6例达R_N，1例达SD_N；血液学疗效评价1例达CR_H，6例达SD_H；血VEGF疗效评价2例达PR_V，5例达SD_V。10例患者中位随访时间48个月（7～123个月），5年OS率70%。结论POEMS综合征起病隐匿，临床表现复杂多样且呈高度异质性，对于不明原因的慢性进展性周围神经病伴皮肤色素沉着应警惕该病，及时进行M蛋白的筛查有助于POEMS综合征的早期诊断。
关键词: POEMS综合征/
临床特征/
周围神经病


Abstract:ObjectiveTo analyze the clinical features, diagnosis and treatment of patients with POEMS syndrome.MethodsWe analyzed the clinical data of 10 patients with POEMS syndrome in the First Affiliated Hospital of Dalian Medical University from March 2012 to April 2021. There were 8 males and 2 females, the median age of onset was 57.5 years old (39-77 years old) and the median time from onset to definite diagnosis was 17 months (4-48 months). We summarized the clinical features, diagnosis, treatment and outcomes.ResultsAmong the 10 patients, 9 patients had chronic onset, 10 patients had peripheral neuropathy, 8 patients had organ enlargement, 8 patients had skin changes, 8 patients had endocrine changes and 2 patients had thrombocytosis. Nine patients could not have a definite diagnosis initially, the median time from the primary to the definite diagnosis was 6 months (1-35 months). Nine patients received antiplasma cell therapy with glucocorticoid alone or in combination with lenalidomide, melphalan or bortezomib. Three patients died of respiratory failure or disease progression. Among the other 7 patients, 7 got R_C in clinical response, 6 got R_N and 1 got SD_N in neuropathy response, 1 got CR_H and 6 got SD_H in hematological response, 2 got PR_V and 5 got SD_V in VEGF response, median follow-up time was 48 months (7-123 months). The overall 5-year survival rate was 70%.ConclusionPOEMS syndrome has insidious onset associated with clinical complexity and high heterogeneity. We should be alert to the unexplained chronic peripheral neuropathy with skin pigmentation, and screening of M protein timely is helpful for the early diagnosis of POEMS syndrome.
Keywords:POEMS syndrome/
clinical features/
peripheral neuropathy

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