Successful treatment of POEMS syndrome with high-dose chemotherapy and autologous peripheral blood stem cell transplantation

摘要

A 42-year-old woman presented with pericardial and pleural effusion, ascites and para-aortic lymphadenopathy of unknown etiology. Six months later she was admitted with fever, pain and motor disturbance of lower limbs, and exacerbation of the effusion, ascites and edema. Physical examination showed hepatosplenomegaly, skin pigmentation and hypertrichosis. Immunoelectophoresis revealed monoclonal IgA-lambda protein in the serum and Bence-Jones protein-lambda in the urine. Bone marrow aspiration showed a mild increase of atypical plasma cells. Vascular endothelial growth factor (VEGF) had markedly increased to 10,900 pg/ml. Electromyography showed changes suggestive of demyelination. These clinical features were consistent with the diagnosis of POEMS syndrome. VAD chemotherapy was not effective for the effusion and neuropathic deterioration. After control of the massive pleural effusion by chest tube drainage, peripheral blood stem cell (PBSC) collection was performed with cyclophosphamide and G-CSF. Thepatient received melphalan 100 mg/m2 on 2 consecutive days and the PBSC were infused 2 days later. The bone marrow recovered rapidly and the pericardial and pleural effusion disappeared completely. Her performance status markedly improved from a bedridden state. High-dose melphalan with auto-PBSCT should be investigated further as a recommended therapy for POEMS syndrome.