[POEMS syndrome]

Abstract

The POEMS syndrome combines a constant polyneuropathy (P), organomegaly (O), endocrinopathy (E), monoclonal gammopathy (M) (or other plasma cell disorder) and skin changes (S). Other manifestations may be observed: anasarca, fever, sweating, clubbed fingers, renal damage, arterial obstruction, pulmonary hypertension, thrombocytosis, and polycythemia. Its pathogenesis is not well elucidated but elevated levels of vascular endothelial growth factor (VEGF) appear to characterize it. Consistent plasma cell disorders include a monoclonal component, often in small quantities with a lambda light chain isotype, and plasmacytoma, often solitary lesions. Treatment depends on specific characteristics of the disease and the patient (radiation therapy for plasmocytoma, autologous bone marrow transplantation in young subjects, corticosteroid therapy or chemotherapy in the elderly). The usefulness of thalidomide and bevacuzimab in refractory POEMS syndromes remains to be seen.