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POEMS Syndrome

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Multiple Myeloma and Other Plasma Cell Neoplasms

Part of the book series: Hematologic Malignancies ((HEMATOLOGIC))

Abstract

POEMS syndrome is a rare paraneoplastic condition due to an underlying plasma cell disorder that presents with a demyelinating peripheral neuropathy. The syndrome is most often associated with osteosclerotic bone lesions and high vascular endothelial growth factor. The other symptoms and signs would appear to be unrelated if one does not consider the diagnosis of this fascinating syndrome. Without adequate treatment, POEMS syndrome is progressive, disabling, and life-threatening. With treatment the prognosis is excellent when drugs from a myeloma armamentarium are employed.

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References

  • Abe D, Nakaseko C, Takeuchi M et al (2008) Restrictive usage of monoclonal immunoglobulin lambda light chain germline in POEMS syndrome. Blood 112:836–839

    Article  CAS  PubMed  Google Scholar 

  • Allam JS, Kennedy CC, Aksamit TR, Dispenzieri A (2008) Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients. Chest 133:969–974

    Article  PubMed  Google Scholar 

  • Authier FJ, Belec L, Levy Y et al (1996) All-trans-retinoic acid in POEMS syndrome. Therapeutic effect associated with decreased circulating levels of proinflammatory cytokines. Arthritis Rheum 39:1423–1426

    Article  CAS  PubMed  Google Scholar 

  • Badros A, Porter N, Zimrin A (2005) Bevacizumab therapy for POEMS syndrome. Blood 106:1135

    Article  CAS  PubMed  Google Scholar 

  • Bardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL (1980) 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 59:311–322

    Article  CAS  PubMed  Google Scholar 

  • Barete S, Mouawad R, Choquet S et al (2010) Skin manifestations and vascular endothelial growth factor levels in POEMS syndrome: impact of autologous hematopoietic stem cell transplantation. Arch Dermatol 146:615–623

    Article  PubMed  Google Scholar 

  • Bryce AH, Ketterling RP, Gertz MA et al (2008) A novel report of cig-FISH and cytogenetics in POEMS syndrome. Am J Hematol 83:840–841

    Article  PubMed  PubMed Central  Google Scholar 

  • Cai QQ, Wang C, Cao XX, Cai H, Zhou DB, Li J (2015) Efficacy and safety of low-dose lenalidomide plus dexamethasone in patients with relapsed or refractory POEMS syndrome. Eur J Haematol 95:325–330

    Article  CAS  PubMed  Google Scholar 

  • Cook G, Iacobelli S, van Biezen A et al (2017) High-dose therapy and autologous stem cell transplantation in patients with POEMS syndrome: a retrospective study of the Plasma Cell Disorder sub-committee of the Chronic Malignancy Working Party of the European Society for Blood & Marrow Transplantation. Haematologica 102:160–167

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Cui R, Yu S, Huang X, Zhang J, Tian C, Pu C (2014) Papilloedema is an independent prognostic factor for POEMS syndrome. J Neurol 261:60–65

    Article  PubMed  Google Scholar 

  • Dao LN, Hanson CA, Dispenzieri A, Morice WG, Kurtin PJ, Hoyer JD (2011) Bone marrow histopathology in POEMS syndrome: a distinctive combination of plasma cell, lymphoid, and myeloid findings in 87 patients. Blood 117:6438–6444

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Dispenzieri A (2007) POEMS syndrome. Blood Rev 21:285–299

    Article  PubMed  Google Scholar 

  • Dispenzieri A (2008) Castleman disease. Cancer Treat Res 142:293–330

    PubMed  Google Scholar 

  • Dispenzieri A (2012) How I treat POEMS syndrome. Blood 119:5650–5658

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Dispenzieri A (2015) POEMS syndrome: update on diagnosis, risk-stratification, and management. Am J Hematol 90:951–962

    Article  CAS  PubMed  Google Scholar 

  • Dispenzieri A, Kyle RA, Lacy MQ et al (2003) POEMS syndrome: definitions and long-term outcome. Blood 101:2496–2506

    Article  CAS  PubMed  Google Scholar 

  • Dispenzieri A, Lacy MQ, Hayman SR et al (2008) Peripheral blood stem cell transplant for POEMS syndrome is associated with high rates of engraftment syndrome. Eur J Haematol 80:397–406

    Article  PubMed  PubMed Central  Google Scholar 

  • D'Souza A, Hayman SR, Buadi F et al (2011) The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome. Blood 118:4663–4665

    Article  PubMed  Google Scholar 

  • D'Souza A, Lacy M, Gertz M et al (2012) Long-term outcomes after autologous stem cell transplantation for patients with POEMS syndrome (osteosclerotic myeloma): a single-center experience. Blood 120:56–62

    Article  PubMed  Google Scholar 

  • Dupont SA, Dispenzieri A, Mauermann ML, Rabinstein AA, Brown RD Jr (2009) Cerebral infarction in POEMS syndrome: incidence, risk factors, and imaging characteristics. Neurology 73:1308–1312

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Gandhi GY, Basu R, Dispenzieri A, Basu A, Montori VM, Brennan MD (2007) Endocrinopathy in POEMS syndrome: the Mayo Clinic experience. Mayo Clin Proc 82:836–842

    Article  PubMed  Google Scholar 

  • He H, Fu W, Du J, Jiang H, Hou J (2017) Successful treatment of newly diagnosed POEMS syndrome with reduced-dose bortezomib based regimen. Br J Haematol. doi:10.1111/bjh.14497. [Epub ahead of print] PubMed PMID: 28146276

  • Hogan WJ, Lacy MQ, Wiseman GA, Fealey RD, Dispenzieri A, Gertz MA (2001) Successful treatment of POEMS syndrome with autologous hematopoietic progenitor cell transplantation. Bone Marrow Transplant 28:305–309

    Article  CAS  PubMed  Google Scholar 

  • Humeniuk MS, Gertz MA, Lacy MQ et al (2013) Outcomes of patients with POEMS syndrome treated initially with radiation. Blood 122:68–73

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Imai N, Taguchi J, Yagi N, Konishi T, Serizawa M, Kobari M (2009) Relapse of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome without increased level of vascular endothelial growth factor following successful autologous peripheral blood stem cell transplantation. Neuromuscul Disord 19:363–365

    Article  PubMed  Google Scholar 

  • Jaccard A, Magy L (2016) Thalidomide and POEMS syndrome: a cautious step forward. Lancet Neurol 15:1104–1105

    Article  PubMed  Google Scholar 

  • Kanai K, Kuwabara S, Misawa S, Hattori T (2007) Failure of treatment with anti-VEGF monoclonal antibody for long-standing POEMS syndrome. Intern Med 46:311–313

    Article  PubMed  Google Scholar 

  • Kanai K, Sawai S, Sogawa K et al (2012) Markedly upregulated serum interleukin-12 as a novel biomarker in POEMS syndrome. Neurology 79:575–582

    Article  CAS  PubMed  Google Scholar 

  • Kang WY, Shen KN, Duan MH et al (2013) 14q32 translocations and 13q14 deletions are common cytogenetic abnormalities in POEMS syndrome. Eur J Haematol 91:490–496

    Article  CAS  PubMed  Google Scholar 

  • Kapoor P, Ansell SM, Fonseca R et al (2017) Diagnosis and management of waldenstrom macroglobulinemia: mayo stratification of macroglobulinemia and risk-adapted therapy (mSMART) guidelines 2016. JAMA Oncol 3(9):1257–1265

    Article  PubMed  PubMed Central  Google Scholar 

  • Katayama K, Misawa S, Sato Y et al (2015) Japanese POEMS syndrome with Thalidomide (J-POST) Trial: study protocol for a phase II/III multicentre, randomised, double-blind, placebo-controlled trial. BMJ Open 5:e007330

    Article  PubMed  PubMed Central  Google Scholar 

  • Kaushik M, Pulido JS, Abreu R, Amselem L, Dispenzieri A (2011) Ocular findings in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome. Ophthalmology 118:778–782

    Article  PubMed  Google Scholar 

  • Kaygusuz I, Tezcan H, Cetiner M, Kocakaya O, Uzay A, Bayik M (2010) Bortezomib: a new therapeutic option for POEMS syndrome. Eur J Haematol 84:175–177

    Article  CAS  PubMed  Google Scholar 

  • Kelly JJ Jr, Kyle RA, Miles JM, Dyck PJ (1983) Osteosclerotic myeloma and peripheral neuropathy. Neurology 33:202–210

    Article  PubMed  Google Scholar 

  • Koga H, Tokunaga Y, Hisamoto T et al (2002) Ratio of serum vascular endothelial growth factor to platelet count correlates with disease activity in a patient with POEMS syndrome. Eur J Intern Med 13:70–74

    Article  CAS  PubMed  Google Scholar 

  • Koike H, Iijima M, Mori K et al (2008) Neuropathic pain correlates with myelinated fibre loss and cytokine profile in POEMS syndrome. J Neurol Neurosurg Psychiatry 79:1171–1179

    Article  CAS  PubMed  Google Scholar 

  • Kojima H, Katsuoka Y, Katsura Y et al (2006) Successful treatment of a patient with POEMS syndrome by tandem high-dose chemotherapy with autologous CD34+ purged stem cell rescue. Int J Hematol 84:182–185

    Article  PubMed  Google Scholar 

  • Kourelis TV, Dispenzieri A (2017) Validation of a prognostic score for patients with POEMS syndrome: a mayo clinic cohort. Leukemia 31:1251

    Article  CAS  PubMed  Google Scholar 

  • Kourelis TV, Buadi FK, Kumar SK et al (2016a) Long-term outcome of patients with POEMS syndrome: an update of the Mayo Clinic experience. Am J Hematol 91:585–589

    Article  CAS  PubMed  Google Scholar 

  • Kourelis TV, Buadi FK, Gertz MA et al (2016b) Risk factors for and outcomes of patients with POEMS syndrome who experience progression after first-line treatment. Leukemia 30:1079–1085

    Article  CAS  PubMed  Google Scholar 

  • Kulkarni GB, Mahadevan A, Taly AB et al (2011) Clinicopathological profile of polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes (POEMS) syndrome. J Clin Neurosci 18:356–360

    Article  CAS  PubMed  Google Scholar 

  • Kuwabara S, Hattori T, Shimoe Y, Kamitsukasa I (1997) Long term melphalan-prednisolone chemotherapy for POEMS syndrome. J Neurol Neurosurg Psychiatry 63:385–387

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Kuwabara S, Misawa S, Kanai K et al (2006) Autologous peripheral blood stem cell transplantation for POEMS syndrome. Neurology 66:105–107

    Article  CAS  PubMed  Google Scholar 

  • Lesprit P, Authier FJ, Gherardi R et al (1996) Acute arterial obliteration: a new feature of the POEMS syndrome? Medicine 75:226–232

    Article  CAS  PubMed  Google Scholar 

  • Lesprit P, Godeau B, Authier FJ et al (1998) Pulmonary hypertension in POEMS syndrome: a new feature mediated by cytokines. Am J Respir Crit Care Med 157:907–911

    Article  CAS  PubMed  Google Scholar 

  • Li J, Zhou DB, Huang Z et al (2011a) Clinical characteristics and long-term outcome of patients with POEMS syndrome in China. Ann Hematol 90:819–826

    Article  PubMed  Google Scholar 

  • Li J, Zhang W, Jiao L et al (2011b) Combination of melphalan and dexamethasone for patients with newly diagnosed POEMS syndrome. Blood 117:6445–6449

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Li J, Zhang W, Kang WY, Cao XX, Duan MH, Zhou DB (2012) 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 53:2527–2529

    Article  CAS  PubMed  Google Scholar 

  • Li J, Tian Z, Zheng HY et al (2013) Pulmonary hypertension in POEMS syndrome. Haematologica 98:393–398

    Article  PubMed  PubMed Central  Google Scholar 

  • Ma L, Wang Y, Bo J et al (2016) Autologous cytokine-induced killer (CIK) cell immunotherapy combined with cyclophosphamide in five patients with POEMS syndrome. Clin Exp Immunol 184:83–89

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Mauermann ML, Sorenson EJ, Dispenzieri A et al (2012) Uniform demyelination and more severe axonal loss distinguish POEMS syndrome from CIDP. J Neurol Neurosurg Psychiatry 83:480–486

    Article  PubMed  Google Scholar 

  • Min JH, Hong YH, Lee KW (2005) Electrophysiological features of patients with POEMS syndrome. Clin Neurophysiol 116:965–968

    Article  PubMed  Google Scholar 

  • Misawa S, Sato Y, Katayama K et al (2016) Safety and efficacy of thalidomide in patients with POEMS syndrome: a multicentre, randomised, double-blind, placebo-controlled trial. Lancet Neurol 15:1129–1137

    Article  CAS  PubMed  Google Scholar 

  • Naddaf E, Dispenzieri A, Mandrekar J, Mauermann ML (2015) Thrombocytosis distinguishes POEMS syndrome from chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 52:658–659

    Article  PubMed  Google Scholar 

  • Nakanishi T, Sobue I, Toyokura Y et al (1984) The Crow-Fukase syndrome: a study of 102 cases in Japan. Neurology 34:712–720

    Article  CAS  PubMed  Google Scholar 

  • Nakano A, Mitsui T, Endo I, Takeda Y, Ozaki S, Matsumoto T (2001) Solitary plasmacytoma with VEGF overproduction: report of a patient with polyneuropathy. Neurology 56:818–819

    Article  CAS  PubMed  Google Scholar 

  • Nasu S, Misawa S, Sekiguchi Y et al (2012) Different neurological and physiological profiles in POEMS syndrome and chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry 83:476–479

    Article  PubMed  Google Scholar 

  • Nishi J, Arimura K, Utsunomiya A et al (1999) Expression of vascular endothelial growth factor in sera and lymph nodes of the plasma cell type of Castleman’s disease. Br J Haematol 104:482–485

    Article  CAS  PubMed  Google Scholar 

  • Nobile-Orazio E, Terenghi F, Giannotta C, Gallia F, Nozza A (2009) Serum VEGF levels in POEMS syndrome and in immune-mediated neuropathies. Neurology 72:1024–1026

    Article  CAS  PubMed  Google Scholar 

  • Ohguchi H, Ohba R, Onishi Y et al (2011) Successful treatment with bortezomib and thalidomide for POEMS syndrome. Ann Hematol 90:1113–1114

    Article  PubMed  Google Scholar 

  • Peggs KS, Paneesha S, Kottaridis PD et al (2002) Peripheral blood stem cell transplantation for POEMS syndrome. Bone Marrow Transplant 30:401–404

    Article  CAS  PubMed  Google Scholar 

  • Roccaro AM, Hideshima T, Raje N et al (2006) Bortezomib mediates antiangiogenesis in multiple myeloma via direct and indirect effects on endothelial cells. Cancer Res 66:184–191

    Article  CAS  PubMed  Google Scholar 

  • Samaras P, Bauer S, Stenner-Liewen F et al (2007) Treatment of POEMS syndrome with bevacizumab. Haematologica 92:1438–1439

    Article  PubMed  Google Scholar 

  • Scarlato M, Previtali SC, Carpo M et al (2005) Polyneuropathy in POEMS syndrome: role of angiogenic factors in the pathogenesis. Brain 128:1911–1920

    Article  PubMed  Google Scholar 

  • Schmitz N, Linch DC, Dreger P et al (1996) Randomised trial of filgrastim-mobilised peripheral blood progenitor cell transplantation versus autologous bone-marrow transplantation in lymphoma patients. Lancet 347:353–357

    Article  CAS  PubMed  Google Scholar 

  • Sekiguchi Y, Misawa S, Shibuya K et al (2013) Ambiguous effects of anti-VEGF monoclonal antibody (bevacizumab) for POEMS syndrome. J Neurol Neurosurg Psychiatry 84:1346–1348

    Article  PubMed  Google Scholar 

  • Shi X, Hu S, Luo X et al (2016) CT characteristics in 24 patients with POEMS syndrome. Acta Radiol 57:51–57

    Article  PubMed  Google Scholar 

  • Singh D, Wadhwa J, Kumar L, Raina V, Agarwal A, Kochupillai V (2003) POEMS syndrome: experience with fourteen cases. Leuk Lymphoma 44:1749–1752

    Article  PubMed  Google Scholar 

  • Soubrier MJ, Dubost JJ, Sauvezie BJ (1994) POEMS syndrome: a study of 25 cases and a review of the literature. French Study Group on POEMS syndrome. Am J Med 97:543–553

    Article  CAS  PubMed  Google Scholar 

  • Soubrier M, Dubost JJ, Serre AF et al (1997) Growth factors in POEMS syndrome: evidence for a marked increase in circulating vascular endothelial growth factor. Arthritis Rheum 40:786–787

    Article  CAS  PubMed  Google Scholar 

  • Soubrier M, Guillon R, Dubost JJ et al (1998) Arterial obliteration in POEMS syndrome: possible role of vascular endothelial growth factor. J Rheumatol 25:813–815

    CAS  PubMed  Google Scholar 

  • Soubrier M, Sauron C, Souweine B et al (1999) Growth factors and proinflammatory cytokines in the renal involvement of POEMS syndrome. Am J Kidney Dis 34:633–638

    Article  CAS  PubMed  Google Scholar 

  • Soubrier M, Ruivard M, Dubost JJ, Sauvezie B, Philippe P (2002) Successful use of autologous bone marrow transplantation in treating a patient with POEMS syndrome. Bone Marrow Transplant 30:61–62

    Article  CAS  PubMed  Google Scholar 

  • Suh YG, Kim YS, Suh CO et al (2014) The role of radiotherapy in the management of POEMS syndrome. Radiat Oncol 9:265

    Article  PubMed  PubMed Central  Google Scholar 

  • Takatsuki K, Sanada I (1983) Plasma cell dyscrasia with polyneuropathy and endocrine disorder: clinical and laboratory features of 109 reported cases. Jpn J Clin Oncol 13:543–555

    CAS  PubMed  Google Scholar 

  • Terracciano C, Fiore S, Doldo E et al (2010) Inverse correlation between VEGF and soluble VEGF receptor 2 in POEMS with AIDP responsive to intravenous immunoglobulin. Muscle Nerve 42:445–448

    Article  PubMed  Google Scholar 

  • Wang C, Zhou YL, Cai H et al (2014) 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 99:e78–e80

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Wang C, Huang XF, Cai QQ et al (2017) Prognostic study for overall survival in patients with newly diagnosed POEMS syndrome. Leukemia 31:100–106

    Article  CAS  PubMed  Google Scholar 

  • Warsame R, Kohut IE, Dispenzieri A (2012) Successful use of cyclophosphamide, bortezomib, and dexamethasone to treat a case of relapsed POEMS. Eur J Haematol 88:549–550

    Article  CAS  PubMed  Google Scholar 

  • Warsame R, Yanamandra U, Kapoor P (2017) POEMS syndrome: an enigma. Curr Hematol Malig Rep 12(2):85–95

    Article  PubMed  Google Scholar 

  • Watanabe O, Arimura K, Kitajima I, Osame M, Maruyama I (1996) Greatly raised vascular endothelial growth factor (VEGF) in POEMS syndrome. Lancet 347:702

    Article  CAS  PubMed  Google Scholar 

  • Zagouri F, Kastritis E, Gavriatopoulou M et al (2014) Lenalidomide in patients with POEMS syndrome: a systematic review and pooled analysis. Leuk Lymphoma 55:2018–2023

    Article  CAS  PubMed  Google Scholar 

  • Zhang B, Song X, Liang B et al (2010) The clinical study of POEMS syndrome in China. Neuro Endocrinol Lett 31:229–237

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Clinical Therapeutics, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece

    Dimitrios C. Ziogas & Evangelos Terpos

  2. Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, MN, USA

    Angela Dispenzieri

Authors
  1. Dimitrios C. Ziogas
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  2. Angela Dispenzieri
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  3. Evangelos Terpos
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Correspondence to Evangelos Terpos .

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Editors and Affiliations

  1. School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

    Meletios A. Dimopoulos

  2. Service des Maladies du Sang, CHRU Lille Hôpital Claude Huriez, Lille, France

    Thierry Facon

  3. School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

    Evangelos Terpos

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Ziogas, D.C., Dispenzieri, A., Terpos, E. (2018). POEMS Syndrome. In: Dimopoulos, M., Facon, T., Terpos, E. (eds) Multiple Myeloma and Other Plasma Cell Neoplasms. Hematologic Malignancies. Springer, Cham. https://doi.org/10.1007/978-3-319-25586-6_10

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