Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

POEMS Syndrome Follow-up

  • Author: Joanna L Chan, MD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Oct 22, 2015
 

Further Outpatient Care

Even if the plasmacytoma completely responds to treatment, patients need long-term follow-up care because some symptoms, such as neurologic defects and functional loss due to tightening of the skin, may be permanent.

Next

Prognosis

The prognosis depends on the extent of the underlying plasma cell disorder and its response to treatment. The prognosis is best for patients with a single lytic lesion. The prognosis is worst for patients with a plasma cell disorder involving the bone marrow. Patients with multiple lytic bone lesions have an intermediate prognosis. When the plasma cell disorder responds to treatment, all other symptoms usually improve or resolve completely. Reduced survival times are associated with the presence of cough, and respiratory weakness decreased mean survival time from 139 months to 87 months in one study.[13]

The morbidity associated with POEMS syndrome depends on the systems involved and ranges from skin pigment alteration to debilitating weakness and loss of function. The median survival period for patients with POEMS syndrome is 8 years. The natural course of POEMS syndrome is chronic, with a reported median survival of approximately a decade (8-13.8 y). In their review, Miralles and colleagues[10] reported a 5-year survival rate of 60%.

Overall shorter survival has been associated with extravascular volume overload (eg, effusions, edema, ascites) and fingernail clubbing. Cardiorespiratory failure, renal failure, infection, and progressive inanition are among the most common causes of death. The neurologic sequelae of POEMS syndrome cause approximately 50% of the patients with POEMS syndrome to become bedridden. Death may also occur as a consequence of decubitus ulcers and thromboses due to inactivity, organomegaly, and endocrinopathy, rather than as a consequence of the aggressiveness of the monoclonal protein.

Previous
 
Contributor Information and Disclosures
Author

Joanna L Chan, MD Mohs Fellow, California Skin Institute

Joanna L Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

Coauthor(s)

Matthew N Kubicki Perelman School of Medicine, University of Pennsylvania

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Neil Shear, MD Professor and Chief of Dermatology, Professor of Medicine, Pediatrics and Pharmacology, University of Toronto Faculty of Medicine; Head of Dermatology, Sunnybrook Women's College Health Sciences Center and Women's College Hospital, Canada

Neil Shear, MD is a member of the following medical societies: Canadian Medical Association, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada, Canadian Dermatology Association, American Academy of Dermatology, American Society for Clinical Pharmacology and Therapeutics

Disclosure: Nothing to disclose.

Wingfield Rehmus, MD, MPH Dermatologist, BC Children's Hospital, Vancouver, British Columbia

Wingfield Rehmus, MD, MPH is a member of the following medical societies: American Academy of Dermatology, Society for Pediatric Dermatology

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Abbvie; Valeant Canada<br/> Received honoraria from Valeant Canada for advisory board; Received honoraria from Pierre Fabre for advisory board; Received honoraria from Mustella for advisory board; Received honoraria from Abbvie for advisory board.

Acknowledgements

Alexa F Boer Kimball, MD, MPH Associate Professor of Dermatology, Harvard University School of Medicine; Vice Chair, Department of Dermatology, Massachusetts General Hospital; Director of Clinical Unit for Research Trials in Skin (CURTIS), Department of Dermatology, Massachusetts General Hospital

Alexa F Boer Kimball, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

References
  1. Ofran Y, Yishay O, Elinav E, Eran E. POEMS syndrome: failure of newly suggested diagnostic criteria to anticipate the development of the syndrome. Am J Hematol. 2005 Aug. 79(4):316-8. [Medline].

  2. Morizane R, Sasamura H, Minakuchi H, Takae Y, Kikuchi H, Yoshiya N, et al. A case of atypical POEMS syndrome without polyneuropathy. Eur J Haematol. 2008 May. 80(5):452-5. [Medline].

  3. Min JH, Hong YH, Lee KW. Electrophysiological features of patients with POEMS syndrome. Clin Neurophysiol. 2005 Apr. 116(4):965-8. [Medline].

  4. Pavord SR, Murphy PT, Mitchell VE. POEMS syndrome and Waldenström's macroglobulinaemia. J Clin Pathol. 1996 Feb. 49(2):181-2. [Medline].

  5. Watanabe O, Arimura K, Kitajima I, Osame M, Maruyama I. Greatly raised vascular endothelial growth factor (VEGF) in POEMS syndrome. Lancet. 1996 Mar 9. 347(9002):702. [Medline].

  6. Kastritis E, Terpos E, Anagnostopoulos A, Xilouri I, Dimopoulos MA. Angiogenetic factors and biochemical markers of bone metabolism in POEMS syndrome treated with high-dose therapy and autologous stem cell support. Clin Lymphoma Myeloma. 2006 Jul. 7(1):73-6. [Medline].

  7. Koike H, Iijima M, Mori K, Yamamoto M, Hattori N, Watanabe H, et al. Neuropathic pain correlates with myelinated fiber loss and cytokine profile in POEMS syndrome. J Neurol Neurosurg Psychiatry. 2008 Apr 3. [Medline].

  8. Lanza C, Misericordia M, Fabrizzi G. POEMS syndrome in a 15-year-old boy: radiological findings. Pediatr Radiol. 2007 Nov. 37(11):1147-50. [Medline].

  9. Sevketoglu E, Hatipoglu S, Ayan I, Dogan O, Salihoglu B. Case report: POEMS syndrome in childhood. J Pediatr Hematol Oncol. 2008 Mar. 30(3):235-8. [Medline].

  10. Miralles GD, O'Fallon JR, Talley NJ. Plasma-cell dyscrasia with polyneuropathy. The spectrum of POEMS syndrome. N Engl J Med. 1992 Dec 31. 327(27):1919-23. [Medline].

  11. Pei G, Yang D, Sun J, Luo Y, Yan J, Chen Y. Cardiac Involvement in a Patient With POEMS Syndrome Detected Using Cardiac Magnetic Resonance Imaging. Int Heart J. 2015 Sep 29. 56 (5):571-3. [Medline].

  12. Gandhi GY, Basu R, Dispenzieri A, Basu A, Montori VM, Brennan MD. Endocrinopathy in POEMS syndrome: the Mayo Clinic experience. Mayo Clin Proc. 2007 Jul. 82(7):836-42. [Medline].

  13. Allam JS, Kennedy CC, Aksamit TR, Dispenzieri A. Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients. Chest. 2008 Apr. 133(4):969-74. [Medline].

  14. Garcia T, Dafer R, Hocker S, Schneck M, Barton K, Biller J. Recurrent strokes in two patients with POEMS syndrome and Castleman's disease. J Stroke Cerebrovasc Dis. 2007 Nov-Dec. 16(6):278-84. [Medline].

  15. Wiaux C, Landau K, Borruat FX. Unusual cause of bilateral optic disc swelling: POEMS syndrome. Klin Monatsbl Augenheilkd. 2007 Apr. 224(4):334-6. [Medline].

  16. Chong DY, Comer GM, Trobe JD. Optic disc edema, cystoid macular edema, and elevated vascular endothelial growth factor in a patient with POEMS syndrome. J Neuroophthalmol. 2007 Sep. 27(3):180-3. [Medline].

  17. Bourgeault E, Dahl A, Thibeault MM, Dupéré A, Drolet AM, Mathieu J. POEMS Syndrome Complicated by Extensive Calciphylaxis: A Remarkable Recovery. J Cutan Med Surg. 2015 May-Jun. 19 (3):309-12. [Medline].

  18. Hudnall SD, Chen T, Brown K, Angel T, Schwartz MR, Tyring SK. Human herpesvirus-8-positive microvenular hemangioma in POEMS syndrome. Arch Pathol Lab Med. 2003 Aug. 127(8):1034-6. [Medline].

  19. Papo T, Soubrier M, Marcelin AG, Calvez V, Wechsler B, Huraux JM, et al. Human herpesvirus 8 infection, Castleman's disease and POEMS syndrome. Br J Haematol. 1999 Mar. 104(4):932-3. [Medline].

  20. Obermoser G, Larcher C, Sheldon JA, Sepp N, Zelger B. Absence of human herpesvirus-8 in glomeruloid haemangiomas associated with POEMS syndrome and Castleman's disease. Br J Dermatol. 2003 Jun. 148(6):1276-8. [Medline].

  21. Colaco SM, Miller T, Ruben BS, Fogarty PF, Fox LP. IgM-lambda paraproteinemia with associated cutaneous lymphoplasmacytic infiltrate in a patient who meets diagnostic criteria for POEMS syndrome. J Am Acad Dermatol. 2008 Apr. 58(4):671-5. [Medline].

  22. Dao LN, Hanson CA, Dispenzieri A, Morice WG, Kurtin PJ, Hoyer JD. Bone marrow histopathology in POEMS syndrome: a distinctive combination of plasma cell, lymphoid and myeloid findings in 87 patients. Blood. 2011 Mar 8. [Medline].

  23. 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 Mar 10. [Medline].

  24. Ganti AK, Pipinos I, Culcea E, Armitage JO, Tarantolo S. Successful hematopoietic stem-cell transplantation in multicentric Castleman disease complicated by POEMS syndrome. Am J Hematol. 2005 Jul. 79(3):206-10. [Medline].

  25. Imai H, Kusuhara S, Nakanishi Y, Teraoka Escaño MF, Yamamoto H, Tsukahara Y, et al. A case of POEMS syndrome with cystoid macular edema. Am J Ophthalmol. 2005 Mar. 139(3):563-6. [Medline].

  26. Giglia F, Chiapparini L, Fariselli L, Barbui T, Ciano C, Scarlato M. POEMS syndrome: relapse after successful autologous peripheral blood stem cell transplantation. Neuromuscul Disord. 2007 Dec. 17(11-12):980-2. [Medline].

  27. Nakaseko C. Autologous stem cell transplantation for POEMS syndrome. Clin Lymphoma Myeloma Leuk. 2014 Feb. 14 (1):21-3. [Medline].

  28. Dispenzieri A, Lacy MQ, Hayman SR, Kumar SK, Buadi F, Dingli D, et al. Peripheral blood stem cell transplant for POEMS syndrome is associated with high rates of engraftment syndrome. Eur J Haematol. 2008 May. 80(5):397-406. [Medline].

  29. Kojima H, Katsuoka Y, Katsura Y, Suzuki S, Suzukawa K, Hasegawa Y, et al. Successful treatment of a patient with POEMS syndrome by tandem high-dose chemotherapy with autologous CD34+ purged stem cell rescue. Int J Hematol. 2006 Aug. 84(2):182-5. [Medline].

  30. Sinisalo M, Hietaharju A, Sauranen J, Wirta O. Thalidomide in POEMS syndrome: case report. Am J Hematol. 2004 May. 76(1):66-8. [Medline].

  31. Badros A, Porter N, Zimrin A. Bevacizumab therapy for POEMS syndrome. Blood. 2005 Aug 1. 106(3):1135. [Medline].

  32. Dietrich PY, Duchosal MA. Bevacizumab therapy before autologous stem-cell transplantation for POEMS syndrome. Ann Oncol. 2008 Mar. 19(3):595. [Medline].

  33. Kanai K, Kuwabara S, Misawa S, Hattori T. Failure of treatment with anti-VEGF monoclonal antibody for long-standing POEMS syndrome. Intern Med. 2007. 46(6):311-3. [Medline].

  34. Straume O, Bergheim J, Ernst P. Bevacizumab therapy for POEMS syndrome. Blood. 2006 Jun 15. 107(12):4972-3; author reply 4973-4. [Medline].

  35. Dispenzieri A, Klein CJ, Mauermann ML. Lenalidomide therapy in a patient with POEMS syndrome. Blood. 2007 Aug 1. 110(3):1075-6. [Medline].

  36. Sethi S, Tageja N, Arabi H, Penumetcha R. Lenalidomide Therapy in a Rare Case of POEMS Syndrome with Kappa Restriction. South Med J. 2009 Sep 4. [Medline].

  37. Jaccard A, Danielou-Lazareth A, Karlin L, Choquet S, Frenzel L, Garderet L. A Prospective Phase II Trial of Lenalidomide and Dexamethasone (Len-Dex) in POEMS Syndrome. Clinical Lymphoma Myeloma and Leukemia. 2015 Sep 27. 15(3):e57. [Full Text].

  38. 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 Apr. 47(4):672-9. [Medline].

  39. Authier FJ, Belec L, Levy Y, Lefaucheur JP, Defer GL, Degos JD, et al. All-trans-retinoic acid in POEMS syndrome. Therapeutic effect associated with decreased circulating levels of proinflammatory cytokines. Arthritis Rheum. 1996 Aug. 39(8):1423-6. [Medline].

  40. Tang X, Shi X, Sun A, et al. Successful bortezomib-based treatment in POEMS syndrome. Eur J Haematol. 2009 Aug 6. [Medline].

  41. Kaygusuz I, Tezcan H, Cetiner M, Kocakaya O, Uzay A, Bayik M. Bortezomib: A New Therapeutic Option for POEMS Syndrome. Eur J Haematol. 2009 Sep 3. [Medline].

  42. Jouve P, Humbert M, Chauveheid MP, Jaïs X, Papo T. POEMS syndrome-related pulmonary hypertension is steroid-responsive. Respir Med. 2007 Feb. 101(2):353-5. [Medline].

  43. Rached S, Athanazio RA, Dias SA Jr, Jardim C, Souza R. Systemic corticosteroids as first-line treatment in pulmonary hypertension associated with POEMS syndrome. J Bras Pneumol. 2009 Aug. 35(8):804-8. [Medline].

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.