POEMS综合征是一种罕见的B细胞异常,具有多种临床体征,包括多发性神经病的首字母缩写,器官肿大,内分泌病,M蛋白和皮肤变化。这是一种副肿瘤综合征,原因是属于临床意义的单克隆丙种球蛋白病(MGCS)的潜在浆细胞疾病。这种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(PCD),硬化骨病变,血管内皮生长因子(VEGF)升高,还有Castleman病的存在.次要特征包括器官肿大,内分泌病,皮肤变化,乳头水肿,血管外容积过载,和血小板增多症。POEMS综合征的诊断需要三个主要标准,其中两个必须包括多发性神经根神经病和克隆性PCD,和至少一个次要标准。VEGF在该疾病中起主要作用,尽管抗VEGF治疗令人失望。风险分层是基于临床表型而不是特定的分子标志物。根据骨髓受累和硬化骨病变的数量,一线治疗应该是放疗或全身治疗.对于患有显性硬化性浆细胞瘤的患者,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,以及在完成放射治疗后3至6个月病情进展的患者,应接受抗浆细胞全身治疗。最有效的是自体干细胞移植的大剂量化疗。来那度胺似乎具有高疗效,毒性可控。沙利度胺和蛋白酶体抑制剂如硼替佐米也是有效的,但需要权衡其获益与加重周围神经病变的风险.
POEMS syndrome is a rare form of B-cell dyscrasia with multiple clinical signs including the acronym for polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes. It is a paraneoplastic syndrome due to an underlying plasma cell disorder belonging to the monoclonal gammopathies of clinical significance (MGCS). The major criteria for this syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor (VEGF), and the presence of Castleman\'s disease. Minor features include organomegaly, endocrinopathy, skin changes, papilledema, extravascular volume over-load, and thrombocytosis. The diagnosis of POEMS syndrome requires three of the major criteria, two of which must include polyradiculoneuropathy and clonal PCD, and at least one of the minor criteria. VEGF plays a major role in the disease although anti-VEGF treatments have been disappointing. Risk stratification is based on clinical phenotype rather than specific molecular markers. Depending on bone marrow involvement and the number of sclerotic bone lesions, first line therapy should be irradiation or systemic therapy. For patients with a dominant sclerotic plasmacytoma, first line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3 to 6 months after completing irradiation therapy should receive antiplasma cell systemic therapy, the most effective being high dose chemotherapy with autologous stem cell transplantation. Lenalidomide seems to have a high efficacy with manageable toxicity. Thalidomide and proteasome inhibitors like bortezomib are also effective, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy.