Coexistence of primary AL amyloidosis and POEMS syndrome.

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes (POEMS) syndrome and primary (AL) amyloidosis are two rare plasma cell disorders with heterogeneous, deceptive clinical features and severe prognosis. With the exception of autologous bone marrow transplantation for selected patients, the best therapeutic approaches are far from being settled and organ-response rates do not exceed 50% in AL amyloidosis and are fairly better in POEMS syndrome associated with localized osteosclerotic myeloma. Moreover, clinical evidence of therapy efficacy is delayed so that biological markers play a crucial role in monitoring diseases course and assessing therapy efficacy. We report on a 57-year-old woman with the unique coexistence of these two plasma cell disorders in whom the close monitoring of serum vascular endothelial growth factor (VEGF) and free light chains (FLC) helped to evaluate the response to melphalan and dexamethasone therapy.