Prognostic study for overall survival in patients with newly diagnosed POEMS syndrome

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) is a multisystem disorder with a good long-term prognosis. In its dozens of clinical features, those with independent prognostic value are still not well characterized. We retrospectively included 362 patients with newly diagnosed POEMS syndrome at our institute from 2000 to 2015. On the basis of a randomized sample splitting, we first identified four baseline clinical variables, including age 450 years (hazards ratio (HR) 4.07, 95% confidence interval (CI) 1.41-11.76, P = 0.009), pulmonary hypertension (HR 3.99, 95% CI 1.44-11.04, P = 0.008), pleural effusion (HR 3.81, 95% CI 1.23-11.79, P = 0.02) and estimated glomerular filtration rate <30 ml/min/1.73 m(2) (HR 8.25, 95% CI 2.18-31.25, P = 0.002), associated with inferior overall survival in the derivation cohort, with the use of multivariate Cox regression model. These factors were incorporated together to develop a prognostic nomogram. Concordance index calculation (0.727, 95% CI 0.601-0.853, P = 0.018) and calibration curve plotting demonstrated its significant predictive and discriminatory capacity in the validation cohort. This nomogram could be a useful and convenient tool in clinical practice to evaluate individualized prognosis in patients with newly diagnosed POEMS syndrome.