Refractory Crow-Fukase Syndrome (POEMS Syndrome) Successfully Treated with High-Dose Melphalan followed by Autologous Peripheral Blood Stem Cell Transplantation

Crow-Fukase syndrome (CFS) is a multisystemic disorder. Because it is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, sclerotic bone lesions, and skin changes it is also known as POEMS syndrome. Extravascular volume overload is also one of the main symptoms. Uncontrollable extravascular volume overload is one of the major causes of death and one of the negative prognostic factors. Control of the extravascular volume overload is an important therapeutic strategy for this syndrome. We report here a case of CFS with extravascular volume overload resulting in pleural effusion and massive edema in the lower extremities, which was refractory to oral administration of melphalan and prednisolone. The patient's condition correlated with the serum level of vascular endothelial growth factor and markedly improved after administration of high-dose melphalan (200 mg/m²) followed by autologous peripheral blood stem cell transplantation. This approach should be considered in patients with CFS who fail to respond to conventional chemotherapy and have uncontrollable extravascular volume overload.