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.
Access optionsAccess options
Subscribe to Journal
Get full journal access for 1 year
only $102.00 per issue
All prices are NET prices.
VAT will be added later in the checkout.
Rent or Buy article
Get time limited or full article access on ReadCube.
All prices are NET prices.
Li J, Zhou DB . New advances in the diagnosis and treatment of POEMS syndrome. Br J Haematol 2013; 161: 303–315.
Dispenzieri A . POEMS syndrome: 2014 update on diagnosis, risk-stratification, and management. Am J Hematol 2014; 89: 214–223.
Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR et al. POEMS syndrome: definitions and long-term outcome. Blood 2003; 101: 2496–2506.
Li J, Zhou DB, Huang Z, Jiao L, Duan MH, Zhang W et al. Clinical characteristics and long-term outcome of patients with POEMS syndrome in China. Ann Hematol 2011; 90: 819–826.
Li J, Tian Z, Zheng HY, Zhang W, Duan MH, Liu YT et al. Pulmonary hypertension in POEMS syndrome. Haematologica 2013; 98: 393–398.
Ye W, Wang C, Cai QQ, Cai H, Duan MH, Li H et al. Renal impairment in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes syndrome: incidence, treatment and outcome. Nephrol Dial Transplant 2015; 31: 275–283, ..
Watanabe O, Arimura K, Kitajima I, Osame M, Maruyama I . Greatly raised vascular endothelial growth factor (VEGF) in POEMS syndrome. Lancet 1996; 347: 702.
D'Souza A, Hayman SR, Buadi F, Mauermann M, Lacy MQ, Gertz MA et al. The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome. Blood 2011; 118: 4663–4665.
Dispenzieri A . How I treat POEMS syndrome. Blood 2012; 119: 5650–5658.
Li J, Zhang W, Jiao L, Duan MH, Guan HZ, Zhu WG et al. Combination of melphalan and dexamethasone for patients with newly diagnosed POEMS syndrome. Blood 2011; 117: 6445–6449.
D'Souza A, Lacy M, Gertz M, Kumar S, Buadi F, Hayman S et al. Long-term outcomes after autologous stem cell transplantation for patients with POEMS syndrome (osteosclerotic myeloma): a single-center experience. Blood 2012; 120: 56–62.
Zagouri F, Kastritis E, Gavriatopoulou M, Sergentanis TN, Psaltopoulou T, Terpos E et al. Lenalidomide in patients with POEMS syndrome: a systematic review and pooled analysis. Leuk Lymphoma 2014; 55: 2018–2023.
Graham RC, Hughes RA . A modified peripheral neuropathy scale: the Overall Neuropathy Limitations Scale. J Neurol Neurosurg Psychiatry 2006; 77: 973–976.
Levey AS, Stevens LA, Schmid CH, Zhang YL, Castro AF 3rd, Feldman HI et al. A new equation to estimate glomerular filtration rate. Ann Intern Med 2009; 150: 604–612.
Wang C, Zhou YL, Cai H, Cheng XQ, Zhang W, Kang WY et al. Markedly elevated serum total N-terminal propeptide of type I collagen is a novel marker for the diagnosis and follow up of patients with POEMS syndrome. Haematologica 2014; 99: e78–e80.
Khorana AA, Kuderer NM, Culakova E, Lyman GH, Francis CW . Development and validation of a predictive model for chemotherapy-associated thrombosis. Blood 2008; 111: 4902–4907.
Eo S-H, Kang HJ, Hong S-M, Cho HY . K-adaptive partitioning for survival data, with an application to cancer staging. 2014; arXiv:1306.4615.
Spyropoulos AC, Anderson Jr FA, Fitzgerald G, Decousus H, Pini M, Chong BH et al. Predictive and associative models to identify hospitalized medical patients at risk for VTE. Chest 2011; 140: 706–714.
Frank E, Harrell Jr . Rms: Regression modeling strategies. R Package version 3.4-0. Available from http://CRAN.R-project.org/package=rms.
Pencina MJ, D'Agostino RB . Overall C as a measure of discrimination in survival analysis: model specific population value and confidence interval estimation. Stat Med 2004; 23: 2109–2123.
Iasonos A, Schrag D, Raj GV, Panageas KS . How to build and interpret a nomogram for cancer prognosis. J Clin Oncol 2008; 26: 1364–1370.
Wang Y, Li J, Xia Y, Gong R, Wang K, Yan Z et al. Prognostic nomogram for intrahepatic cholangiocarcinoma after partial hepatectomy. J Clin Oncol 2013; 31: 1188–1195.
Yang Y, Zhang YJ, Zhu Y, Cao JZ, Yuan ZY, Xu LM et al. Prognostic nomogram for overall survival in previously untreated patients with extranodal NK/T-cell lymphoma, nasal-type: a multicenter study. Leukemia 2015; 29: 1571–1577.
Allam JS, Kennedy CC, Aksamit TR, Dispenzieri A . Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients. Chest 2008; 133: 969–974.
Cui R, Yu S, Huang X, Zhang J, Tian C, Pu C . Papilloedema is an independent prognostic factor for POEMS syndrome. J Neurol 2014; 261: 60–65.
Humeniuk MS, Gertz MA, Lacy MQ, Kyle RA, Witzig TE, Kumar SK et al. Outcomes of patients with POEMS syndrome treated initially with radiation. Blood 2013; 122: 68–73.
Clegg A, Young J, Iliffe S, Rikkert MO, Rockwood K . Frailty in elderly people. Lancet 2013; 381: 752–762.
Lesprit P, Godeau B, Authier FJ, Soubrier M, Zuber M, Larroche C et al. Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines. Am J Respir Crit Care Med 1998; 157 (3 Pt 1): 907–911.
Nakamoto Y, Imai H, Yasuda T, Wakui H, Miura AB . A spectrum of clinicopathological features of nephropathy associated with POEMS syndrome. Nephrol Dial Transplant 1999; 14: 2370–2378.
Sanada S, Ookawara S, Karube H, Shindo T, Goto T, Nakamichi T et al. Marked recovery of severe renal lesions in POEMS syndrome with high-dose melphalan therapy supported by autologous blood stem cell transplantation. Am J Kidney Dis 2006; 47: 672–679.
Shimizu N, Nakaseko C, Sakaida E, Ohwada C, Takeuchi M, Kawaguchi T et al. Factors associated with the efficiency of PBSC collection in POEMS syndrome patients undergoing autologous PBSC transplantation. Bone Marrow Transplant 2012; 47: 1010–1012.
Li J, Zhang W, Duan MH, Jiao L, Zhu TN, Han B et al. PBSC mobilization in newly diagnosed patients with POEMS syndrome: outcomes and prognostic factors. Bone Marrow Transplant 2013; 48: 233–237.
Kourelis TV, Buadi FK, Gertz MA, Lacy MQ, Kumar SK, Kapoor P et al. Risk factors for and outcomes of patients with POEMS syndrome who experience progression after first-line treatment. Leukemia 2015; 30: 1079–1085.
Nakanishi T, Sobue I, Toyokura Y, Nishitani H, Kuroiwa Y, Satoyoshi E et al. The Crow-Fukase syndrome: a study of 102 cases in Japan. Neurology 1984; 34: 712–720.
Wang C, Su W, Cai QQ, Cai H, Ji W, Di Q et al. Prognostic value of serum heavy/light chain ratios in patients with POEMS syndrome. Eur J Haematol 2015; 97: 48–54.
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.
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.
The authors declare no conflict of interest.
Supplementary Information accompanies this paper on the Leukemia website
About this article
Clinical Lymphoma Myeloma and Leukemia (2019)
Frontiers in Immunology (2019)
American Journal of Hematology (2019)
High‐dose therapy and autologous transplantation for POEMS Syndrome: effective, but how to optimise?
British Journal of Haematology (2019)
British Journal of Haematology (2019)