POEMS syndrome: monoclonal plasma cell disorder with polyneuropathy

Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a rare multi-organ disease. Because the peripheral neuropathy is frequently the overriding symptom and because the characteristics of the neuropathy are similar to that chronic inflammatory demyelinating polyneuropathy(CIDP), patient are frequently misdiagnosed with CIDP or monoclonal gammopathy of undetermined significance (MGUS) -associated peripheral neuropathy. Production of vascular endothelial growth factor (VEGF), secreted by plasmacytoma, is considered responsible for these symptoms. For isolated plasmacytoma, radiotherapy is usually highly effective. Patients without isolated plasmacytoma require systemic chemotherapy, which is ineffective in many cases. In recent reports, high dose chemotherapy followed by autologous peripheral blood stem cell transplantation can dramatically improve clinical manifestations, particularly for polyneuropathy in such patients refractory for standard chemotherapy.