Department of Hematology, Marmara University School of Medicine, Marmara University, Istanbul, Turkey.
Eur J Haematol. 2010 Feb 1;84(2):175-7. doi: 10.1111/j.1600-0609.2009.01341.x. Epub 2009 Sep 3.
POEMS syndrome with its classical five findings (Polyneuropathy, Organomegaly, Endocrinopathy, M protein, and Skin changes) is a rare multisystem disease. Proinflammatory and proangiogenic cytokines play important roles in its pathogenesis. Treatment options are still debated.
We present a 65-year-old man with POEMS syndrome who was successfully treated with bortezomib.
After seven cycles of this protocol, serum M protein level declined to normal range, and near-to-complete remission was achieved. His symptoms of polyneuropathy improved dramatically.
Bortezomib may be an effective and safe therapeutic option for patients with POEMS syndrome.
POEMS 综合征具有其经典的“五联征”(多发性神经病、脏器肿大、内分泌病、M 蛋白和皮肤改变),是一种罕见的多系统疾病。促炎和促血管生成细胞因子在其发病机制中起重要作用。治疗方案仍存在争议。
我们报告了一例成功应用硼替佐米治疗的 POEMS 综合征患者。
该患者接受七周期硼替佐米治疗后,血清 M 蛋白水平降至正常范围,达到近乎完全缓解。其多发性神经病症状显著改善。
硼替佐米可能是 POEMS 综合征患者的一种有效且安全的治疗选择。