Gandhi GY et al. (2007) Endocrinopathy in POEMS syndrome: the Mayo Clinic experience. Mayo Clin Proc 82: 836–842

A study was performed by Gandhi et al. to determine the prevalence and characteristics of endocrinopathies in POEMS syndrome, a rare disorder characterized by polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes. The diagnosis of the disorder is based on the presence of two major criteria (polyneuropathy, clonal plasma cell proliferative disorder) and at least one minor criterion (sclerotic bone lesions, Castleman disease, organomegaly, edema, endocrinopathy, skin changes, papilledema).

In this study the medical records of 170 patients diagnosed with POEMS syndrome were analyzed retrospectively. Patients seen in or after 2000 (n = 64) were found to have had a more-complete endocrine evaluation and results were, therefore, focused on these patients.

Out of the 64 patients seen in or after 2000, 84% had an endocrinopathy. Seventy-nine percent of men, whose testosterone levels were measured, had levels below the normal range. Prolactin levels were higher than normal in 29% out of 35 patients. In 58% out of 48 patients, thyrotropin levels were elevated. Glucose metabolism was abnormal in 48% out of 50 patients. Adrenal insufficiency was found in 67% out of nine patients tested. Among 51 patients, 27% had true hypocalcemia. Multiple endocrinopathies were found in 54% of patients.

The authors suggest that the high prevalence of endocrinopathies in POEMS syndrome should lead clinicians to perform a thorough endocrine evaluation in patients diagnosed with this syndrome, and to monitor patients for endocrine abnormalities emerging during the course of the disease.