The multisystem disorder POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a rare plasma cell dyscrasia. Elevated cytokines may contribute to its pathogenesis. We describe a POEMS patient with elevated plasma cytokines and excessive daytime sleepiness (EDS).
A 39-year-old white woman with IgA lambda and IgG kappa paraproteinaemia, peripheral axonal sensorimotor neuropathy, moderate anascara, skin thickening and pigmentation, hepatosplenomegaly, premature ovarian failure, and idiopathic hyperprolactinaemia, was treated with multiple pulses of intravenous steroids and plasma exchange followed by an autologous stem transplant. All POEMS features improved, although IgG kappa paraproteinaemia remained. However, 2 years later she presented with EDS. Mean plasma cytokines over a 4 hour period (09 00 - 1300 h: taken half hourly) were elevated: TNF-alpha (51.9 ± 5.5 vs 13.9 ± 3.9; mean ± SEM, compared with 20 normal controls,); interleukin-6 (IL-6; 9.45 ± 1.9 vs 1.5 ± 0.5) and IL-1beta (3.9 ± 1.3 vs 1.1 ± 0.4). Serum leptin, adiponectin and resistin were similar to controls. Multiple sleep latency testing (MSLT) following overnight polysommography confirmed severe hypersommia with a very short latency to enter deep, phase IV, non-rapid eye movement sleep. Her body mass index was 20.4. Obstructive sleep apnoea was absent.
High cytokine levels including IL-6, TNF-alpha, and IL-1beta, may contribute to EDS in various neurological disorders. To our knowledge, this is the first description of hypersomnia associated with hypercytokinaemia in POEMS. Our findings suggest that cytokine-lowering therapies may be useful in treating this novel presentation of the syndrome.
22 - 24 Mar 2004
British Endocrine Societies