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POEMS syndrome

S Vincent Rajkumar, MD
Section Editor
Robert A Kyle, MD
Deputy Editor
Rebecca F Connor, MD


POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes) is characterized by the presence of a monoclonal plasma cell disorder, peripheral neuropathy, and one or more of the following features: osteosclerotic myeloma, Castleman's disease (angiofollicular lymph node hyperplasia), increased levels of serum vascular endothelial growth factor (VEGF), organomegaly, endocrinopathy, edema, typical skin changes, and papilledema [1].

The clinical features, diagnosis, and treatment of POEMS syndrome will be discussed here. A discussion of Castleman's disease is presented separately. (See "Multicentric Castleman's disease".)


In 1938, a patient was described with sensorimotor peripheral neuropathy, hyperpigmentation, elevated cerebrospinal fluid protein, and a solitary plasmacytoma [2]. Eighteen years later, two patients were reported with peripheral neuropathy, hyperpigmentation, elevation of cerebrospinal fluid protein, and plasmacytomas with new bone formation [3]. In 1980, the acronym "POEMS" was suggested for this disorder, representing the following constellation of findings [4]:



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Literature review current through: Nov 2017. | This topic last updated: Sep 22, 2017.
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  1. Dispenzieri A, Kyle RA, Lacy MQ, et al. POEMS syndrome: definitions and long-term outcome. Blood 2003; 101:2496.
  2. Scheinker I. Myelom und Nervensystem: über eine bisher nicht beschriebene mit eigentümlichen Hautveränderungen einhergehende Polyneuritis bei einem plasmazellulären myelom des Sternums. Dtsch Z Nervenheilkd 1938; 147:247.
  3. CROW RS. Peripheral neuritis in myelomatosis. Br Med J 1956; 2:802.
  4. Bardwick PA, Zvaifler NJ, Gill GN, et al. Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature. Medicine (Baltimore) 1980; 59:311.
  5. Gherardi RK, Bélec L, Soubrier M, et al. Overproduction of proinflammatory cytokines imbalanced by their antagonists in POEMS syndrome. Blood 1996; 87:1458.
  6. Lesprit P, Godeau B, Authier FJ, et al. Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines. Am J Respir Crit Care Med 1998; 157:907.
  7. Scarlato M, Previtali SC, Carpo M, et al. Polyneuropathy in POEMS syndrome: role of angiogenic factors in the pathogenesis. Brain 2005; 128:1911.
  8. Hitoshi S, Suzuki K, Sakuta M. Elevated serum interleukin-6 in POEMS syndrome reflects the activity of the disease. Intern Med 1994; 33:583.
  9. Shikama N, Isono A, Otsuka Y, et al. A case of POEMS syndrome with high concentrations of interleukin-6 in pericardial fluid. J Intern Med 2001; 250:170.
  10. Hashiguchi T, Arimura K, Matsumuro K, et al. Highly concentrated vascular endothelial growth factor in platelets in Crow-Fukase syndrome. Muscle Nerve 2000; 23:1051.
  11. Endo I, Mitsui T, Nishino M, et al. Diurnal fluctuation of edema synchronized with plasma VEGF concentration in a patient with POEMS syndrome. Intern Med 2002; 41:1196.
  12. Shinde A, Matsumae H, Maruyama A, et al. [A patient with Crow-Fukase syndrome associated with pulmonary plasmacytoma]. Rinsho Shinkeigaku 2001; 41:121.
  13. Watanabe O, Maruyama I, Arimura K, et al. Overproduction of vascular endothelial growth factor/vascular permeability factor is causative in Crow-Fukase (POEMS) syndrome. Muscle Nerve 1998; 21:1390.
  14. Kuwabara S, Misawa S, Kanai K, et al. Autologous peripheral blood stem cell transplantation for POEMS syndrome. Neurology 2006; 66:105.
  15. Michizono K, Umehara F, Hashiguchi T, et al. Circulating levels of MMP-1, -2, -3, -9, and TIMP-1 are increased in POEMS syndrome. Neurology 2001; 56:807.
  16. D'Souza A, Hayman SR, Buadi F, 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.
  17. Sung JY, Kuwabara S, Ogawara K, et al. Patterns of nerve conduction abnormalities in POEMS syndrome. Muscle Nerve 2002; 26:189.
  18. Ono K, Ito M, Hotchi M, et al. Polyclonal plasma cell proliferation with systemic capillary hemangiomatosis, endocrine disturbance, and peripheral neuropathy. Acta Pathol Jpn 1985; 35:251.
  19. Adams D, Said G. Ultrastructural characterisation of the M protein in nerve biopsy of patients with POEMS syndrome. J Neurol Neurosurg Psychiatry 1998; 64:809.
  20. Nakanishi T, Sobue I, Toyokura Y, et al. The Crow-Fukase syndrome: a study of 102 cases in Japan. Neurology 1984; 34:712.
  21. Abe D, Nakaseko C, Takeuchi M, et al. Restrictive usage of monoclonal immunoglobulin lambda light chain germline in POEMS syndrome. Blood 2008; 112:836.
  22. Soubrier MJ, Dubost JJ, Sauvezie BJ. POEMS syndrome: a study of 25 cases and a review of the literature. French Study Group on POEMS Syndrome. Am J Med 1994; 97:543.
  23. Narváez JA, Majós C, Narváez J, et al. POEMS syndrome: unusual radiographic, scintigraphic and CT features. Eur Radiol 1998; 8:134.
  24. Shibuya K, Misawa S, Horikoshi T, et al. Detection of bone lesions by CT in POEMS syndrome. Intern Med 2011; 50:1393.
  25. D'Souza A, Lacy M, Gertz M, 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.
  26. Montoriol PF, Cachin F, Michel JL, Soubrier M. Two more cases of evaluation of POEMS syndrome using 18-FDG PET/CT. Eur J Radiol 2011; 80:861.
  27. Stefanelli A, Treglia G, Leccisotti L, et al. Usefulness of F-18 FDG PET/CT in the follow-up of POEMS syndrome after autologous peripheral blood stem cell transplantation. Clin Nucl Med 2012; 37:181.
  28. Albertí MA, Martinez-Yélamos S, Fernandez A, et al. 18F-FDG PET/CT in the evaluation of POEMS syndrome. Eur J Radiol 2010; 76:180.
  29. Dao LN, Hanson CA, Dispenzieri A, et al. Bone marrow histopathology in POEMS syndrome: a distinctive combination of plasma cell, lymphoid, and myeloid findings in 87 patients. Blood 2011; 117:6438.
  30. Kanai K, Sawai S, Sogawa K, et al. Markedly upregulated serum interleukin-12 as a novel biomarker in POEMS syndrome. Neurology 2012; 79:575.
  31. Webb NJ, Bottomley MJ, Watson CJ, Brenchley PE. Vascular endothelial growth factor (VEGF) is released from platelets during blood clotting: implications for measurement of circulating VEGF levels in clinical disease. Clin Sci (Lond) 1998; 94:395.
  32. Bitter MA, Komaiko W, Franklin WA. Giant lymph node hyperplasia with osteoblastic bone lesions and the POEMS (Takatsuki's) syndrome. Cancer 1985; 56:188.
  33. Gherardi R, Baudrimont M, Kujas M, et al. Pathological findings in three non-Japanese patients with the POEMS syndrome. Virchows Arch A Pathol Anat Histopathol 1988; 413:357.
  34. Bélec L, Mohamed AS, Authier FJ, et al. Human herpesvirus 8 infection in patients with POEMS syndrome-associated multicentric Castleman's disease. Blood 1999; 93:3643.
  35. Li J, Zhou DB, Huang Z, et al. Clinical characteristics and long-term outcome of patients with POEMS syndrome in China. Ann Hematol 2011; 90:819.
  36. Gandhi GY, Basu R, Dispenzieri A, et al. Endocrinopathy in POEMS syndrome: the Mayo Clinic experience. Mayo Clin Proc 2007; 82:836.
  37. Shichiri M, Iwashina M, Imai T, et al. Abnormal FSH hypersecretion as an endocrinological manifestation of POEMS syndrome. Endocr J 1998; 45:131.
  38. Perniciaro C. POEMS syndrome. Semin Dermatol 1995; 14:162.
  39. Tsai CY, Lai CH, Chan HL, Kuo Tt . Glomeruloid hemangioma--a specific cutaneous marker of POEMS syndrome. Int J Dermatol 2001; 40:403.
  40. Miest RY, Comfere NI, Dispenzieri A, et al. Cutaneous manifestations in patients with POEMS syndrome. Int J Dermatol 2013; 52:1349.
  41. Kelly JJ Jr, Kyle RA, Miles JM, Dyck PJ. Osteosclerotic myeloma and peripheral neuropathy. Neurology 1983; 33:202.
  42. Modesto-Segonds A, Rey JP, Orfila C, et al. Renal involvement in POEMS syndrome. Clin Nephrol 1995; 43:342.
  43. Soubrier M, Sauron C, Souweine B, et al. Growth factors and proinflammatory cytokines in the renal involvement of POEMS syndrome. Am J Kidney Dis 1999; 34:633.
  44. Nakamoto Y, Imai H, Yasuda T, et al. A spectrum of clinicopathological features of nephropathy associated with POEMS syndrome. Nephrol Dial Transplant 1999; 14:2370.
  45. Lambotte O, Dürrbach A, Ammor M, et al. Association of a POEMS syndrome and light chain deposit disease: first case report. Clin Nephrol 2001; 55:482.
  46. Lesprit P, Authier FJ, Gherardi R, et al. Acute arterial obliteration: a new feature of the POEMS syndrome? Medicine (Baltimore) 1996; 75:226.
  47. Ichikawa Y, Nakata T, Ohhata J, et al. Crow-Fukase syndrome with ischemic cardiomyopathy. Intern Med 2001; 40:726.
  48. Saida K, Kawakami H, Ohta M, Iwamura K. Coagulation and vascular abnormalities in Crow-Fukase syndrome. Muscle Nerve 1997; 20:486.
  49. Dupont SA, Dispenzieri A, Mauermann ML, et al. Cerebral infarction in POEMS syndrome: incidence, risk factors, and imaging characteristics. Neurology 2009; 73:1308.
  50. Rose C, Zandecki M, Copin MC, et al. POEMS syndrome: report on six patients with unusual clinical signs, elevated levels of cytokines, macrophage involvement and chromosomal aberrations of bone marrow plasma cells. Leukemia 1997; 11:1318.
  51. Ribadeau-Dumas S, Tillie-Leblond I, Rose C, et al. Pulmonary hypertension associated with POEMS syndrome. Eur Respir J 1996; 9:1760.
  52. 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.
  53. Kyle RA, Rajkumar SV. Criteria for diagnosis, staging, risk stratification and response assessment of multiple myeloma. Leukemia 2009; 23:3.
  54. Rajkumar SV, Dimopoulos MA, Palumbo A, et al. International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol 2014; 15:e538.
  55. Morizane R, Sasamura H, Minakuchi H, et al. A case of atypical POEMS syndrome without polyneuropathy. Eur J Haematol 2008; 80:452.
  56. Ofran Y, Elinav E. POEMS syndrome: failure of newly suggested diagnostic criteria to anticipate the development of the syndrome. Am J Hematol 2005; 79:316.
  57. Charli-Joseph Y, Fernández-Sánchez M, Saeb-Lima M, Orozco-Topete R. POEMS syndrome: are current diagnostic criteria too exclusive? J Am Acad Dermatol 2011; 65:415.
  58. Eurelings M, Notermans NC, Van de Donk NW, Lokhorst HM. Risk factors for hematological malignancy in polyneuropathy associated with monoclonal gammopathy. Muscle Nerve 2001; 24:1295.
  59. Vital A. Paraproteinemic neuropathies. Brain Pathol 2001; 11:399.
  60. Wicklund MP, Kissel JT. Paraproteinemic Neuropathy. Curr Treat Options Neurol 2001; 3:147.
  61. Gorson KC. Clinical features, evaluation, and treatment of patients with polyneuropathy associated with monoclonal gammopathy of undetermined significance (MGUS). J Clin Apher 1999; 14:149.
  62. Lacy MQ, Gertz MA, Hanson CA, et al. Multiple myeloma associated with diffuse osteosclerotic bone lesions: a clinical entity distinct from osteosclerotic myeloma (POEMS syndrome). Am J Hematol 1997; 56:288.
  63. Lipsker D, Rondeau M, Massard G, Grosshans E. The AESOP (adenopathy and extensive skin patch overlying a plasmacytoma) syndrome: report of 4 cases of a new syndrome revealing POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome at a curable stage. Medicine (Baltimore) 2003; 82:51.
  64. Néel A, Hello M, Barbarot S, et al. Skin patch, polyneuropathy, and paraproteinemia. Am J Med 2012; 125:e1.
  65. Nobile-Orazio E, Casellato C, Di Troia A. Neuropathies associated with IgG and IgA monoclonal gammopathy. Rev Neurol (Paris) 2002; 158:979.
  66. Fisher MA, Wilson JR. Characterizing neuropathies associated with monoclonal gammopathy of undetermined significance (MGUS): a framework consistent with classifying injuries according to fiber size. Neurol Clin Neurophysiol 2002; 2002:2.
  67. Mauermann ML, Sorenson EJ, Dispenzieri A, et al. Uniform demyelination and more severe axonal loss distinguish POEMS syndrome from CIDP. J Neurol Neurosurg Psychiatry 2012; 83:480.
  68. Nasu S, Misawa S, Sekiguchi Y, et al. Different neurological and physiological profiles in POEMS syndrome and chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry 2012; 83:476.
  69. Dispenzieri A. How I treat POEMS syndrome. Blood 2012; 119:5650.
  70. Kuwabara S, Dispenzieri A, Arimura K, et al. Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome. Cochrane Database Syst Rev 2012; :CD006828.
  71. Humeniuk MS, Gertz MA, Lacy MQ, et al. Outcomes of patients with POEMS syndrome treated initially with radiation. Blood 2013; 122:68.
  72. Dispenzieri A. POEMS syndrome: 2017 Update on diagnosis, risk stratification, and management. Am J Hematol 2017; 92:814.
  73. Li J, Zhang W, Jiao L, et al. Combination of melphalan and dexamethasone for patients with newly diagnosed POEMS syndrome. Blood 2011; 117:6445.
  74. Dispenzieri A, Klein CJ, Mauermann ML. Lenalidomide therapy in a patient with POEMS syndrome. Blood 2007; 110:1075.
  75. Tomás JF, Giraldo P, Lecumberri R, Nistal S. POEMS syndrome with severe neurological damage clinically recovered with lenalidomide. Haematologica 2012; 97:320.
  76. Royer B, Merlusca L, Abraham J, et al. Efficacy of lenalidomide in POEMS syndrome: a retrospective study of 20 patients. Am J Hematol 2013; 88:207.
  77. Briani C, Manara R, Lessi F, et al. Pachymeningeal involvement in POEMS syndrome: dramatic cerebral MRI improvement after lenalidomide therapy. Am J Hematol 2012; 87:539.
  78. Sethi S, Tageja N, Arabi H, Penumetcha R. Lenalidomide therapy in a rare case of POEMS syndrome with kappa restriction. South Med J 2009; 102:1092.
  79. Suyanı E, Yağcı M, Sucak GT. Complete remission with a combination of lenalidomide, cyclophosphamide and prednisolone in a patient with incomplete POEMS syndrome. Acta Haematol 2011; 126:199.
  80. Vannata B, Laurenti L, Chiusolo P, et al. Efficacy of lenalidomide plus dexamethasone for POEMS syndrome relapsed after autologous peripheral stem-cell transplantation. Am J Hematol 2012; 87:641.
  81. Zagouri F, Kastritis E, Gavriatopoulou M, et al. Lenalidomide in patients with POEMS syndrome: a systematic review and pooled analysis. Leuk Lymphoma 2014; 55:2018.
  82. Kuwabara S, Misawa S, Kanai K, et al. Thalidomide reduces serum VEGF levels and improves peripheral neuropathy in POEMS syndrome. J Neurol Neurosurg Psychiatry 2008; 79:1255.
  83. Warsame R, Kohut IE, Dispenzieri A. Successful use of cyclophosphamide, bortezomib, and dexamethasone to treat a case of relapsed POEMS. Eur J Haematol 2012; 88:549.
  84. Li J, Zhang W, Kang WY, et al. Bortezomib and dexamethasone as first-line therapy for a patient with newly diagnosed polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes syndrome complicated by renal failure. Leuk Lymphoma 2012; 53:2527.
  85. Zeng K, Yang JR, Li J, et al. Effective induction therapy with subcutaneous administration of bortezomib for newly diagnosed POEMS syndrome: a case report and a review of the literature. Acta Haematol 2013; 129:101.
  86. He H, Fu W, Du J, et al. Successful treatment of newly diagnosed POEMS syndrome with reduced-dose bortezomib based regimen. Br J Haematol 2017.
  87. Authier FJ, Belec L, Levy Y, et al. All-trans-retinoic acid in POEMS syndrome. Therapeutic effect associated with decreased circulating levels of proinflammatory cytokines. Arthritis Rheum 1996; 39:1423.
  88. Badros A, Porter N, Zimrin A. Bevacizumab therapy for POEMS syndrome. Blood 2005; 106:1135.
  89. Straume O, Bergheim J, Ernst P. Bevacizumab therapy for POEMS syndrome. Blood 2006; 107:4972.
  90. Dietrich PY, Duchosal MA. Bevacizumab therapy before autologous stem-cell transplantation for POEMS syndrome. Ann Oncol 2008; 19:595.
  91. Karam C, Klein CJ, Dispenzieri A, et al. Polyneuropathy improvement following autologous stem cell transplantation for POEMS syndrome. Neurology 2015; 84:1981.
  92. Jaccard A, Royer B, Bordessoule D, et al. High-dose therapy and autologous blood stem cell transplantation in POEMS syndrome. Blood 2002; 99:3057.
  93. Rovira M, Carreras E, Bladé J, et al. Dramatic improvement of POEMS syndrome following autologous haematopoietic cell transplantation. Br J Haematol 2001; 115:373.
  94. Kuwabara S, Misawa S, Kanai K, et al. Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome. Neurology 2008; 71:1691.
  95. Graham RC, Hughes RA. A modified peripheral neuropathy scale: the Overall Neuropathy Limitations Scale. J Neurol Neurosurg Psychiatry 2006; 77:973.