Smarter Decisions,
Better Care

UpToDate synthesizes the most recent medical information into evidence-based practical recommendations clinicians trust to make the right point-of-care decisions.

  • Rigorous editorial process: Evidence-based treatment recommendations
  • World-Renowned physician authors: over 5,100 physician authors and editors around the globe
  • Innovative technology: integrates into the workflow; access from EMRs

Choose from the list below to learn more about subscriptions for a:


Subscribers log in here


POEMS syndrome

INTRODUCTION

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".)

HISTORICAL BACKGROUND

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]:

Polyneuropathy

Organomegaly

                                             

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Jun 2014. | This topic last updated: Apr 28, 2014.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2014 UpToDate, Inc.
References
Top
  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. 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. Morizane R, Sasamura H, Minakuchi H, et al. A case of atypical POEMS syndrome without polyneuropathy. Eur J Haematol 2008; 80:452.
  55. 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.
  56. 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.
  57. 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.
  58. Vital A. Paraproteinemic neuropathies. Brain Pathol 2001; 11:399.
  59. Wicklund MP, Kissel JT. Paraproteinemic Neuropathy. Curr Treat Options Neurol 2001; 3:147.
  60. 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.
  61. 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.
  62. 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.
  63. Néel A, Hello M, Barbarot S, et al. Skin patch, polyneuropathy, and paraproteinemia. Am J Med 2012; 125:e1.
  64. Nobile-Orazio E, Casellato C, Di Troia A. Neuropathies associated with IgG and IgA monoclonal gammopathy. Rev Neurol (Paris) 2002; 158:979.
  65. 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.
  66. 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.
  67. 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.
  68. Dispenzieri A. How I treat POEMS syndrome. Blood 2012; 119:5650.
  69. 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; 6:CD006828.
  70. Humeniuk MS, Gertz MA, Lacy MQ, et al. Outcomes of patients with POEMS syndrome treated initially with radiation. Blood 2013; 122:68.
  71. Li J, Zhang W, Jiao L, et al. Combination of melphalan and dexamethasone for patients with newly diagnosed POEMS syndrome. Blood 2011; 117:6445.
  72. Dispenzieri A, Klein CJ, Mauermann ML. Lenalidomide therapy in a patient with POEMS syndrome. Blood 2007; 110:1075.
  73. Tomás JF, Giraldo P, Lecumberri R, Nistal S. POEMS syndrome with severe neurological damage clinically recovered with lenalidomide. Haematologica 2012; 97:320.
  74. 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.
  75. 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.
  76. 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.
  77. 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.
  78. 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.
  79. 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.
  80. 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.
  81. 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.
  82. 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.
  83. 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.
  84. Badros A, Porter N, Zimrin A. Bevacizumab therapy for POEMS syndrome. Blood 2005; 106:1135.
  85. Straume O, Bergheim J, Ernst P. Bevacizumab therapy for POEMS syndrome. Blood 2006; 107:4972.
  86. Dietrich PY, Duchosal MA. Bevacizumab therapy before autologous stem-cell transplantation for POEMS syndrome. Ann Oncol 2008; 19:595.
  87. Jaccard A, Royer B, Bordessoule D, et al. High-dose therapy and autologous blood stem cell transplantation in POEMS syndrome. Blood 2002; 99:3057.
  88. Rovira M, Carreras E, Bladé J, et al. Dramatic improvement of POEMS syndrome following autologous haematopoietic cell transplantation. Br J Haematol 2001; 115:373.
  89. Kuwabara S, Misawa S, Kanai K, et al. Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome. Neurology 2008; 71:1691.
  90. Graham RC, Hughes RA. A modified peripheral neuropathy scale: the Overall Neuropathy Limitations Scale. J Neurol Neurosurg Psychiatry 2006; 77:973.