POEMS综合征是一种罕见的单克隆浆细胞疾病,其临床特征为多发性神经病、脏器肿大、内分泌病变、单克隆免疫球蛋白血症以及皮肤改变。腹泻症状在POEMS综合征中并不常见。我们报告了一例以腹泻为主要表现的复发性POEMS综合征患者的病例。...POEMS综合征是一种罕见的单克隆浆细胞疾病,其临床特征为多发性神经病、脏器肿大、内分泌病变、单克隆免疫球蛋白血症以及皮肤改变。腹泻症状在POEMS综合征中并不常见。我们报告了一例以腹泻为主要表现的复发性POEMS综合征患者的病例。该患者接受了多次胃镜检查和息肉切除术,但症状并未改善。随后的免疫固定电泳显示存在IgA-λ型M蛋白条带,且血管内皮生长因子升高,提示可能存在POEMS综合征复发。腹泻作为POEMS综合征罕见且不典型的症状,给POEMS综合征复发的诊断带来了重大挑战。全面评估和管理对于POEMS综合征及早诊断及改善预后具有重要作用。POEMS syndrome is a rare monoclonal plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Diarrhea is an uncommon manifestation in POEMS syndrome. We report a case of recurrent POEMS syndrome presenting predominantly with diarrhea. The patient underwent multiple gastroscopies and polypectomies without symptomatic improvement. Subsequent immunofixation electrophoresis revealed an IgA-λ monoclonal protein band, and elevated vascular endothelial growth factor (VEGF) levels suggested potential disease recurrence. Diarrhea, as a rare and atypical symptom of POEMS syndrome, posed significant diagnostic challenges in identifying disease relapse. Comprehensive evaluation and management are critical for early diagnosis and improving prognosis in POEMS syndrome.展开更多
POEMS(polyneuropathy,organomegaly,endocrinopathy,M-protein and skin changes)综合征是一种单克隆浆细胞异常增殖的副肿瘤综合征,患病率约为0.3/10万[1]。该病累积神经、内分泌、骨骼、免疫及造血等系统,临床上诊断需要2个基本要素...POEMS(polyneuropathy,organomegaly,endocrinopathy,M-protein and skin changes)综合征是一种单克隆浆细胞异常增殖的副肿瘤综合征,患病率约为0.3/10万[1]。该病累积神经、内分泌、骨骼、免疫及造血等系统,临床上诊断需要2个基本要素:多发性周围神经病和单克隆浆细胞病[2]。周围神经病变是超过60%的POEMS综合征患者的首发症状,不少患者早期就诊于神经内科并被误诊为慢性炎性脱髓鞘性多发性神经根神经病(chronic inflammatory demyelinating polyneuropathy,CIDP)等原发性神经系统疾病[3]。由于神经病变进展缓慢、致残率高,多数患者就诊时症状严重且已造成神经不可逆性损伤,给患者生活带来极大不便,因此早识别、早诊断、及时治疗至关重要。本文报告1例采用达雷妥尤单抗(Daratumumab,Dara)联合方案治疗伴有严重周围神经病变的POEMS综合征,分析了神经病变的发病机制,并强调了POEMS综合征诊断的复杂性以及Daratumumab作为新一代药物在治疗中的潜在优势。展开更多
文摘POEMS综合征是一种罕见的单克隆浆细胞疾病,其临床特征为多发性神经病、脏器肿大、内分泌病变、单克隆免疫球蛋白血症以及皮肤改变。腹泻症状在POEMS综合征中并不常见。我们报告了一例以腹泻为主要表现的复发性POEMS综合征患者的病例。该患者接受了多次胃镜检查和息肉切除术,但症状并未改善。随后的免疫固定电泳显示存在IgA-λ型M蛋白条带,且血管内皮生长因子升高,提示可能存在POEMS综合征复发。腹泻作为POEMS综合征罕见且不典型的症状,给POEMS综合征复发的诊断带来了重大挑战。全面评估和管理对于POEMS综合征及早诊断及改善预后具有重要作用。POEMS syndrome is a rare monoclonal plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Diarrhea is an uncommon manifestation in POEMS syndrome. We report a case of recurrent POEMS syndrome presenting predominantly with diarrhea. The patient underwent multiple gastroscopies and polypectomies without symptomatic improvement. Subsequent immunofixation electrophoresis revealed an IgA-λ monoclonal protein band, and elevated vascular endothelial growth factor (VEGF) levels suggested potential disease recurrence. Diarrhea, as a rare and atypical symptom of POEMS syndrome, posed significant diagnostic challenges in identifying disease relapse. Comprehensive evaluation and management are critical for early diagnosis and improving prognosis in POEMS syndrome.
