内容紹介
Summary
A man in his late 40s was presented to a hospital with complaints of peripheral numbness and fatigue. Systemic edema, pleural effusion and ascites, pigmentation, splenomegaly, and CT findings of osteoplastic changes suggested POEMS syndrome. He was referred to our division, and a bone marrow examination indicated MGUS. However, his serum level of vascular endothelial growth factor(VEGF)was elevated to 1,520 pg/mL, and IgA-λ type M protein was detected. He was diagnosed with POEMS syndrome and received four cycles of induction chemotherapy containing lenalidomide and dexamethasone(Ld). All symptoms improved gradually, and after auto peripheral blood stem cell harvest(aPBSCH), high-dose melphalan was administered, followed by auto peripheral blood stem cell transplantation(aPBSCT)being performed. Pleural effusion and ascites disappeared, while numbness remained slightly. His serum level of VEGF decreased to 68 pg/mL when the planned primary treatment was completed. Many cases of POEMS syndrome involve peripheral neuropathy; therefore, a lenalidomide-containing regimen may be a more adequate strategy than ones containing thalidomide and bortezomib.
要旨
患者は40歳台後半,男性。末梢神経障害,疲労感で前医初診し,全身浮腫,胸腹水,皮膚色素沈着,脾腫,硬化性骨病変があり,POEMS症候群が疑われた。当科紹介となり,骨髄穿刺ではmonoclonal gammopathy with undetermined significance(MGUS)であったが,血清血管内皮増殖因子(VEGF)1,529 pg/mLと高値であり,IgA-λ型M蛋白を検出した。POEMS症候群と診断され,Ld 4コースの寛解導入療法後,大量melphalan(L-PAM)療法を施行した。軽度のしびれは残存するも胸腹水は消失し,VEGF 68 pg/mLと減少した。多くの患者において末梢神経障害を有することから,thalidomide(THAL)やbortezomib(BOR)に比してlenalidomide(LEN)を含むレジメンが適している可能性が考えられた。
目次
A man in his late 40s was presented to a hospital with complaints of peripheral numbness and fatigue. Systemic edema, pleural effusion and ascites, pigmentation, splenomegaly, and CT findings of osteoplastic changes suggested POEMS syndrome. He was referred to our division, and a bone marrow examination indicated MGUS. However, his serum level of vascular endothelial growth factor(VEGF)was elevated to 1,520 pg/mL, and IgA-λ type M protein was detected. He was diagnosed with POEMS syndrome and received four cycles of induction chemotherapy containing lenalidomide and dexamethasone(Ld). All symptoms improved gradually, and after auto peripheral blood stem cell harvest(aPBSCH), high-dose melphalan was administered, followed by auto peripheral blood stem cell transplantation(aPBSCT)being performed. Pleural effusion and ascites disappeared, while numbness remained slightly. His serum level of VEGF decreased to 68 pg/mL when the planned primary treatment was completed. Many cases of POEMS syndrome involve peripheral neuropathy; therefore, a lenalidomide-containing regimen may be a more adequate strategy than ones containing thalidomide and bortezomib.
要旨
患者は40歳台後半,男性。末梢神経障害,疲労感で前医初診し,全身浮腫,胸腹水,皮膚色素沈着,脾腫,硬化性骨病変があり,POEMS症候群が疑われた。当科紹介となり,骨髄穿刺ではmonoclonal gammopathy with undetermined significance(MGUS)であったが,血清血管内皮増殖因子(VEGF)1,529 pg/mLと高値であり,IgA-λ型M蛋白を検出した。POEMS症候群と診断され,Ld 4コースの寛解導入療法後,大量melphalan(L-PAM)療法を施行した。軽度のしびれは残存するも胸腹水は消失し,VEGF 68 pg/mLと減少した。多くの患者において末梢神経障害を有することから,thalidomide(THAL)やbortezomib(BOR)に比してlenalidomide(LEN)を含むレジメンが適している可能性が考えられた。