Laboratory Medicine and Pathology, Division of Hematology, Mayo Clinic, Rochester, MN 55905, USA.
Am J Hematol. 2011 Jul;86(7):591-601. doi: 10.1002/ajh.22050.
POEMS syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), 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. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy. POEMS syndrome should be distinguished from the Castleman disease variant of POEMS syndrome, which has no clonal PCD and typically little to no peripheral neuropathy but has several of the minor diagnostic criteria for POEMS syndrome.
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. The number of clinical criteria is not prognostic, but the extent of the plasma cell disorder is. Those patients with an iliac crest bone marrow biopsy that does not reveal a plasma cell clone are candidates for local radiation therapy; those with a more extensive or disseminated clone will be candidates for systemic therapy.
RISK-ADAPTED THERAPY: For those 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 radiation therapy should receive systemic therapy. Corticosteroids are temporizing, but alkylators are the mainstay of treatment, either in the form of low dose conventional therapy or high dose with stem cell transplantation. The benefit of anti-VEGF antibodies is conflicting. Lenalidomide shows promise with manageable toxicity. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes.
POEMS 综合征是一种副肿瘤综合征，由潜在的浆细胞瘤引起。该综合征的主要标准为多发性神经根神经病、克隆性浆细胞疾病（PCD）、硬化性骨病变、血管内皮生长因子升高以及伴发的 Castleman 病。次要特征包括器官肿大、内分泌病、特征性皮肤改变、视乳头水肿、血管外容量超负荷和血小板增多症。由于该综合征较为罕见，且可能被误诊为其他神经疾病，最常见的是慢性炎症性脱髓鞘性多发性神经病，因此诊断常常被延误。POEMS 综合征应与 Castleman 病变异型 POEMS 综合征相区分，后者无克隆性 PCD，通常很少或没有周围神经病，但具有 POEMS 综合征的一些次要诊断标准。
POEMS 综合征的诊断需要满足三个主要标准中的三个，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，并且至少满足一个次要标准。
由于该综合征的发病机制尚未完全阐明，因此风险分层仅限于临床表型而非特定的分子标志物。临床标准的数量并不具有预后意义，但浆细胞疾病的程度具有预后意义。那些髂骨骨髓活检未发现浆细胞克隆的患者适合接受局部放射治疗；那些有更广泛或弥散性克隆的患者将成为系统治疗的候选者。
对于那些有显性硬化性浆细胞瘤的患者，一线治疗是放疗。对于弥漫性硬化性病变或弥散性骨髓受累的患者，以及那些在完成放射治疗后 3 至 6 个月疾病进展的患者，应接受系统治疗。皮质类固醇只是暂时缓解症状，但烷化剂是治疗的主要药物，无论是低剂量常规治疗还是高剂量联合干细胞移植。抗血管内皮生长因子（VEGF）抗体的疗效存在争议。来那度胺具有良好的疗效且毒性可管理。沙利度胺和硼替佐米也具有活性，但需要权衡其导致周围神经病恶化的风险。及时识别并采取支持性护理措施和针对浆细胞的治疗可获得最佳效果。
