内容紹介
A Case of POEMS Syndrome Responding to Autologous Peripheral Blood Stem Cell Transplantation
Summary
The patient, a 44-year-old male, subjectively noticed edema in his lower legs in March, 2010. By June 2010, he could not walk, and was admitted to this hospital. Since multiple neuropathy, increased serum vascular endothelial growth factor-3, M-proteinemia, edema, pericardial effusions, papilledema, increased platelet count and skin lesions were detected in the complete examination, he was diagnosed with polyneuropathy, organomegaly, endocrinopathy, M-proteinemia and skin changes(POEMS)syndrome. Treatment with steroid and furosemide was initiated in September 2010. This markedly improved edema and brought about mild recovery of proximal lower-limb muscle strength. The patient, however, suffered prolonged difficulty in walking because his distal lower-limb muscle strength was not improved. In October 2010, he received treatment with high-dose cyclophosphamide, followed by peripheral blood stem cell sampling with granulocyte-colony stimulating factor. In November 2010, he underwent treatment with high-dose melphalan, followed by autologous peripheral blood stem cell transplantation. Recovery to neutrophils greater than 500 was achieved at 13 days after transplantation. He could walk without assistance in February 2011. M-protein was not identified by immunofixation electrophoresis in August 2011. Autologous peripheral blood stem cell transplantation is regarded as a useful treatment for POEMS syndrome.
要旨
患者は44歳,男性。2010年3月に両側下腿の浮腫を自覚,6月から歩行困難となったため,当院に入院となった。精査の結果,多発ニューロパチー,血清vascular endothelial growth factor-3高値,M蛋白血症,浮腫,心嚢液貯留,うっ血乳頭,血小板増加および皮膚病変を認めたため,われわれは本症例をPOEMS症候群と診断した。9月よりステロイド剤ならびにフロセミドの投与を開始したところ,浮腫は著明に改善,下肢近位筋の筋力も軽度回復した。しかしながら下肢遠位筋の筋力は改善せず,歩行困難が続いた。10月にcyclophosphamide大量療法を行った後,顆粒球コロニー増加因子を用いて末梢血幹細胞採取を行った。11月にmelphalan大量療法を施行した後,自家末梢血幹細胞移植を施行した。移植後13日目に好中球は500以上に回復した。2011年2月には自立歩行が可能となった。8月にはM蛋白は免疫固定法で陰性化した。自家末梢血幹細胞移植はPOEMS症候群に対して有用な治療法であるものと考えられる。
目次
Summary
The patient, a 44-year-old male, subjectively noticed edema in his lower legs in March, 2010. By June 2010, he could not walk, and was admitted to this hospital. Since multiple neuropathy, increased serum vascular endothelial growth factor-3, M-proteinemia, edema, pericardial effusions, papilledema, increased platelet count and skin lesions were detected in the complete examination, he was diagnosed with polyneuropathy, organomegaly, endocrinopathy, M-proteinemia and skin changes(POEMS)syndrome. Treatment with steroid and furosemide was initiated in September 2010. This markedly improved edema and brought about mild recovery of proximal lower-limb muscle strength. The patient, however, suffered prolonged difficulty in walking because his distal lower-limb muscle strength was not improved. In October 2010, he received treatment with high-dose cyclophosphamide, followed by peripheral blood stem cell sampling with granulocyte-colony stimulating factor. In November 2010, he underwent treatment with high-dose melphalan, followed by autologous peripheral blood stem cell transplantation. Recovery to neutrophils greater than 500 was achieved at 13 days after transplantation. He could walk without assistance in February 2011. M-protein was not identified by immunofixation electrophoresis in August 2011. Autologous peripheral blood stem cell transplantation is regarded as a useful treatment for POEMS syndrome.
要旨
患者は44歳,男性。2010年3月に両側下腿の浮腫を自覚,6月から歩行困難となったため,当院に入院となった。精査の結果,多発ニューロパチー,血清vascular endothelial growth factor-3高値,M蛋白血症,浮腫,心嚢液貯留,うっ血乳頭,血小板増加および皮膚病変を認めたため,われわれは本症例をPOEMS症候群と診断した。9月よりステロイド剤ならびにフロセミドの投与を開始したところ,浮腫は著明に改善,下肢近位筋の筋力も軽度回復した。しかしながら下肢遠位筋の筋力は改善せず,歩行困難が続いた。10月にcyclophosphamide大量療法を行った後,顆粒球コロニー増加因子を用いて末梢血幹細胞採取を行った。11月にmelphalan大量療法を施行した後,自家末梢血幹細胞移植を施行した。移植後13日目に好中球は500以上に回復した。2011年2月には自立歩行が可能となった。8月にはM蛋白は免疫固定法で陰性化した。自家末梢血幹細胞移植はPOEMS症候群に対して有用な治療法であるものと考えられる。