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POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome is an uncommon disorder of plasma cells that also results in extravascular volume overload and osteosclerosis. The optimum approach to managing patients with advanced disease is unclear.
To address this issue, investigators in Japan now report long-term outcomes of 36 patients (median age, 49 years; range, 16–65) with rapidly progressive POEMS who were given induction therapy — including thalidomide in 70%, lenalidomide in 14%, and bortezomib in 14% — followed by high-dose melphalan and ASCT.
Results were as follows:
5-year clinical progression-free survival was 63%, and 5-year overall survival was 90%.
Mean vascular endothelial growth factor (VE…