POEMS and calciphylaxis: a novel association?

characterized by polyneuropathy (P), organomegaly (O), endocrinopathy (E), monoclonal gammopathy (M) and skin abnormalities (S)5. POEMS syndrome is a rare disease, since its prevalence, although still not well defined, has been estimated to be < 1% in cases of monoclonal gammopathies1,3. However, the true prevalence of POEMS syndrome may be underestimated, since no single test establishes the diagnosis, but a high clinical suspicion is necessary to link the disparate signs and symptoms that characterize the disease. Recently, researchers at the Mayo Clinic revised the clinical criteria for the diagnosis of POEMS syndrome on the bases of a large mono-institutional series, and suggested that polyneuropathy and plasma cell disorder, associated with at least one additional minor criteria (Table I), are necessary for the diagnosis2. The heterogeneity and complexity of the presenting features of POEMS syndrome are also documented by the existence of several clinical conditions which, although without diagnostic relevance based on the above-mentioned criteria, have been found to be associated to POEMS syndrome2. Activation of the proinflammatory cytokine network has been implicated in the pathogenesis of POEMS syndrome. Indeed, serum levels of interleukin (IL)-1β, IL-6 and tumor necrosis factor (TNF)-α are increased in patients affected by POEMS syndrome and a correlation has been found between cytokine levels and disease activity6,7. Proinflammatory cytokines may be the causative agents of the characteristic hallmarks of the syndrome, including endocrine abnormalities, skin changes, constitutional symptoms, edema, effusions, clubbing and organomegaly6,7. The site of proinflammatory cytokine production is unknown, nevertheless some lines of evidence suggest that the microenvironment, rather than the plasma cell clone, is responsible for the production6,7. This scenario appears to closely mimic multiple myeloma, in which cytokine production is mainly ascribed to stromal and accessory cells in the microenvironment rather than to the plasma cell population itself. U.D.A. Ematologia (Responsabile: Prof. Gianluca Gaidano), Dipartimento di Scienze Mediche & IRCAD, Università degli Studi del Piemonte Orientale “Amedeo Avogadro” di Novara © 2004 CEPI Srl (Ann Ital Med Int 2004; 19: 235-237)