Multiple Myeloma, Gammopathies

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

Subjects

Abstract

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 >50 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 m2 (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.

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Acknowledgements

We thank all the patients who participated in this study. We extend our appreciation to all physicians caring for these patients. Dr Zhuang Tao provided valuable statistical review. Dr Yijia Li gave some insightful suggestions. The National Natural Science Foundation of China (grant No. 81570195), the Beijing Natural Science Foundation (grant No. 7142130), the Specialized Research Fund for the Doctoral Program of Higher Education (grant No. 2013110611000), and the Peking Union Medical College New Star (2011, for LJ) supported this research.

Author contributions

CW was involved in study design, data collection and analysis, and writing the manuscript. X-FH, Q-QC, X-XC and M-HD participated in patient recruitment. HC measured serum VEGF. D-BZ supervised the study and analyzed the data. JL designed the study, recruited the patients, analyzed the data, and wrote and critically revised the manuscript. All authors have approved the final manuscript.

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Correspondence to J Li.

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The authors declare no conflict of interest.

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Supplementary Information accompanies this paper on the Leukemia website

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