Abstract：
POEMS syndrome is a life-threatening condition due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder, sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis.
The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria.
Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced estimated glomerular filtration rate.
For those patients with a dominant plasmacytoma, first-line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement should receive systemic therapy. Corticosteroids are temporizing, but alkylators and lenalidomide are the mainstays of treatment, the former either in the form of low-dose conventional therapy or as high-dose conditioning for stem cell transplantation. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. Daratumumab combinations also appear promising based on case series. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes.
The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, and at least one of the minor criteria.
Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced estimated glomerular filtration rate.
For those patients with a dominant plasmacytoma, first-line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement should receive systemic therapy. Corticosteroids are temporizing, but alkylators and lenalidomide are the mainstays of treatment, the former either in the form of low-dose conventional therapy or as high-dose conditioning for stem cell transplantation. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. Daratumumab combinations also appear promising based on case series. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes.
摘要：
背景：由于潜在的浆细胞肿瘤，POEMS综合征是一种危及生命的疾病。该综合征的主要标准是多发性神经根神经病，克隆浆细胞疾病，硬化骨病变，血管内皮生长因子升高，还有Castleman病的存在.次要特征包括器官肿大，内分泌病,特征性皮肤变化,乳头水肿,血管外容量超负荷,和血小板增多症。
方法：POEMS综合征的诊断有三个主要标准，其中两个必须包括多发性神经根神经病和克隆性浆细胞疾病，和至少一个次要标准。
方法：由于该综合征的发病机制尚未完全了解，风险分层仅限于临床表型，而不是特定的分子标志物。危险因素包括低血清白蛋白，年龄,胸腔积液,肺动脉高压,并降低估计的肾小球滤过率。
方法：对于患有显性浆细胞瘤的患者，一线治疗是放疗。弥漫性硬化病变或播散性骨髓受累的患者应接受全身治疗。皮质类固醇是暂时的，但是烷化剂和来那度胺是治疗的支柱，前者以低剂量常规治疗或高剂量预处理的形式用于干细胞移植。沙利度胺和硼替佐米也有活性，但需要权衡其获益与加重周围神经病变的风险.根据病例系列，Daratumumab组合似乎也很有希望。及时识别和建立针对浆细胞的支持性护理措施和治疗可获得最佳结果。
方法：POEMS综合征的诊断有三个主要标准，其中两个必须包括多发性神经根神经病和克隆性浆细胞疾病，和至少一个次要标准。
方法：由于该综合征的发病机制尚未完全了解，风险分层仅限于临床表型，而不是特定的分子标志物。危险因素包括低血清白蛋白，年龄,胸腔积液,肺动脉高压,并降低估计的肾小球滤过率。
方法：对于患有显性浆细胞瘤的患者，一线治疗是放疗。弥漫性硬化病变或播散性骨髓受累的患者应接受全身治疗。皮质类固醇是暂时的，但是烷化剂和来那度胺是治疗的支柱，前者以低剂量常规治疗或高剂量预处理的形式用于干细胞移植。沙利度胺和硼替佐米也有活性，但需要权衡其获益与加重周围神经病变的风险.根据病例系列，Daratumumab组合似乎也很有希望。及时识别和建立针对浆细胞的支持性护理措施和治疗可获得最佳结果。
