eMedicine Specialties > Dermatology > Internal Medicine

POEMS Syndrome: Follow-up

Author: Joanna L Chan, MD, Resident Physician, Department of Dermatology, University of Texas Southwestern Medical Center
Coauthor(s): Wingfield Rehmus, MD, MPH, ; 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
Contributor Information and Disclosures

Updated: Sep 25, 2009

Follow-up

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.

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.12

 


More on POEMS Syndrome

Overview: POEMS Syndrome
Differential Diagnoses & Workup: POEMS Syndrome
Treatment & Medication: POEMS Syndrome
Follow-up: POEMS Syndrome
References
[ CLOSE WINDOW ]

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. Aug 2005;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. May 2008;80(5):452-5. [Medline].

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

  4. Pavord SR, Murphy PT, Mitchell VE. POEMS syndrome and Waldenström's macroglobulinaemia. J Clin Pathol. Feb 1996;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. Mar 9 1996;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. Jul 2006;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. Apr 3 2008;[Medline].

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

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

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

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

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

  13. 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. Nov-Dec 2007;16(6):278-84. [Medline].

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

  15. 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. Sep 2007;27(3):180-3. [Medline].

  16. 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. Aug 2003;127(8):1034-6. [Medline].

  17. 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. Mar 1999;104(4):932-3. [Medline].

  18. 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. Jun 2003;148(6):1276-8. [Medline].

  19. 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. Apr 2008;58(4):671-5. [Medline].

  20. 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. Jul 2005;79(3):206-10. [Medline].

  21. 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. Mar 2005;139(3):563-6. [Medline].

  22. 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. Dec 2007;17(11-12):980-2. [Medline].

  23. 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. May 2008;80(5):397-406. [Medline].

  24. 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. Aug 2006;84(2):182-5. [Medline].

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

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

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

  28. 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].

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

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

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

  32. 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. Apr 2006;47(4):672-9. [Medline].

  33. 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. Aug 1996;39(8):1423-6. [Medline].

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

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

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

  37. 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. Aug 2009;35(8):804-8. [Medline].

  38. Cabezas-Agricola JM, Lado-Abeal JJ, Otero-Anton E, Sanchez-Leira J, Cabezas-Cerrato J. Hypoparathyroidism in POEMS syndrome. Lancet. Mar 9 1996;347(9002):701-2. [Medline].

  39. Calonje E, Wilson-Jones E. Vascular tumors. In: Elder D, Elenitsas R, Jaworsky C, eds. Lever's Histopathology of the Skin. 8th ed. Philadelphia, Pa: Lippincott-Raven; 1997:890-1.

  40. Di Napoli M, Arakelyan A. POEMS syndrome, fibrinogen, and ischemic stroke: a critical point of view. Arch Neurol. Jan 2004;61(1):155; author reply 155-6. [Medline].

  41. Dispenzieri A, Gertz MA. Treatment options for POEMS syndrome. Expert Opin Pharmacother. Jun 2005;6(6):945-53. [Medline].

  42. Dispenzieri A, Kyle RA. Neurological aspects of multiple myeloma and related disorders. Best Pract Res Clin Haematol. 2005;18(4):673-88. [Medline].

  43. Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR, et al. POEMS syndrome: definitions and long-term outcome. Blood. Apr 1 2003;101(7):2496-506. [Medline].

  44. Dispenzieri A, Moreno-Aspitia A, Suarez GA, Lacy MQ, Colon-Otero G, Tefferi A, et al. Peripheral blood stem cell transplantation in 16 patients with POEMS syndrome, and a review of the literature. Blood. Nov 15 2004;104(10):3400-7. [Medline].

  45. Donofrio PD. Immunotherapy of idiopathic inflammatory neuropathies. Muscle Nerve. Sep 2003;28(3):273-92. [Medline].

  46. Erro ME, Lacruz F, Aymerich N, Ayuso T, Soriano G, Gállego J, et al. Acute carotid obliteration: a new vascular manifestation in POEMS syndrome. Eur J Neurol. Jul 2003;10(4):383-4. [Medline].

  47. Fishel B, Brenner S, Weiss S, Yaron M. POEMS syndrome associated with cryoglobulinemia, lymphoma, multiple seborrheic keratosis, and ichthyosis. J Am Acad Dermatol. Nov 1988;19(5 Pt 2):979-82. [Medline].

  48. Goebels N, Walther EU, Schaller M, Pongratz D, Mueller-Felber W. Inflammatory myopathy in POEMS syndrome. Neurology. Nov 14 2000;55(9):1413-4. [Medline].

  49. Imai N, Kitamura E, Tachibana T, Konishi T, Suzuki Y, Serizawa M. Efficacy of autologous peripheral blood stem cell transplantation in POEMS syndrome with polyneuropathy. Intern Med. 2007;46(3):135-8. [Medline].

  50. Kang K, Chu K, Kim DE, Jeong SW, Lee JW, Roh JK. POEMS syndrome associated with ischemic stroke. Arch Neurol. May 2003;60(5):745-9. [Medline].

  51. Kim SK, Park IK, Park BH, Park W, Lee HS, Kim TH, et al. A case report: isolated a heavy chain monoclonal gammopathy in a patient with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin change syndrome. Int J Clin Pract Suppl. Apr 2005;(147):26-30. [Medline].

  52. Kuwabara S, Hattori T, Shimoe Y, Kamitsukasa I. Long term melphalan-prednisolone chemotherapy for POEMS syndrome. J Neurol Neurosurg Psychiatry. Sep 1997;63(3):385-7. [Medline].

  53. Kuwabara S, Misawa S, Kanai K, Kikkawa Y, Nishimura M, Nakaseko C, et al. Autologous peripheral blood stem cell transplantation for POEMS syndrome. Neurology. Jan 10 2006;66(1):105-7. [Medline].

  54. Mokhlesi B, Jain M. Pulmonary manifestations of POEMS syndrome: case report and literature review. Chest. Jun 1999;115(6):1740-2. [Medline].

  55. Samaras P, Bauer S, Stenner-Liewen F, Steiner R, Zweifel M, Renner C, et al. Treatment of POEMS syndrome with bevacizumab. Haematologica. Oct 2007;92(10):1438-9. [Medline].

  56. Shibata M, Yamada T, Tanahashi N, Koto A, Kuramochi S, Fukushima S, et al. POEMS syndrome with necrotizing vasculitis: a novel feature of vascular abnormalities. Neurology. Feb 8 2000;54(3):772-3. [Medline].

  57. Soubrier M, Dubost JJ, Serre AF, Ristori JM, Sauvezie B, Cathebras P, et al. Growth factors in POEMS syndrome: evidence for a marked increase in circulating vascular endothelial growth factor. Arthritis Rheum. Apr 1997;40(4):786-7. [Medline].

  58. 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. Dec 1994;97(6):543-53. [Medline].

  59. Vélez D, Delgado-Jiménez Y, Fraga J. Solitary glomeruloid haemangioma without POEMS syndrome. J Cutan Pathol. Jul 2005;32(6):449-52. [Medline].

  60. Warren KJ. POEMS syndrome in a patient with diabetic ketoacidosis: case report and review. Cutis. Jun 1998;61(6):329-34. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

Crow-Fukase syndrome, Takatsuki syndrome, PEP, polyneuropathy endocrinopathy plasma cell dyscrasia, polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes, Castleman disease, Castleman's disease, Takatsuki syndrome

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Joanna L Chan, MD, Resident Physician, Department of Dermatology, University of Texas Southwestern Medical Center
Joanna L Chan, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Dermatologic Surgery
Disclosure: Nothing to disclose.

Coauthor(s)

Wingfield Rehmus, MD, MPH, 
Wingfield Rehmus, MD, MPH is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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.

Medical Editor

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: American Academy of Dermatology, American Society for Clinical Pharmacology and Therapeutics, Canadian Dermatology Association, Canadian Medical Association, Ontario Medical Association, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Warren R Heymann, MD, Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey
Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.