poems syndrome

Summary

Summary: A multisystemic disorder characterized by a sensorimotor polyneuropathy (POLYNEUROPATHIES), organomegaly, endocrinopathy, monoclonal gammopathy, and pigmentary skin changes. Other clinical features which may be present include EDEMA; CACHEXIA; microangiopathic glomerulopathy; pulmonary hypertension (HYPERTENSION, PULMONARY); cutaneous necrosis; THROMBOCYTOSIS; and POLYCYTHEMIA. This disorder is frequently associated with osteosclerotic myeloma. (From Adams et al., Principles of Neurology, 6th ed, p1335; Rev Med Interne 1997;18(7):553-62)

Top Publications

  1. Dispenzieri A, Kyle R, Lacy M, Rajkumar S, Therneau T, Larson D, et al. POEMS syndrome: definitions and long-term outcome. Blood. 2003;101:2496-506 pubmed
    The POEMS syndrome (coined to refer to polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) remains poorly understood...
  2. Dispenzieri A, Gertz M. Treatment options for POEMS syndrome. Expert Opin Pharmacother. 2005;6:945-53 pubmed
    b>POEMS syndrome is a rare paraneoplastic syndrome secondary to a plasma cell dyscrasia...
  3. Gherardi R, Belec L, Soubrier M, Malapert D, Zuber M, Viard J, et al. Overproduction of proinflammatory cytokines imbalanced by their antagonists in POEMS syndrome. Blood. 1996;87:1458-65 pubmed
    ..TNF receptors (sTNFr) p55 and p75, and soluble IL-6 receptor (sIL-6r) were determined in 15 patients with POEMS syndrome and 15 with multiple myeloma...
  4. Barete S, Mouawad R, Choquet S, Viala K, Leblond V, Musset L, et al. Skin manifestations and vascular endothelial growth factor levels in POEMS syndrome: impact of autologous hematopoietic stem cell transplantation. Arch Dermatol. 2010;146:615-23 pubmed publisher
  5. Li J, Zhang W, Jiao L, Duan M, Guan H, Zhu W, et al. Combination of melphalan and dexamethasone for patients with newly diagnosed POEMS syndrome. Blood. 2011;117:6445-9 pubmed publisher
    b>POEMS syndrome is a rare clonal plasma cell disorder without standard treatment...
  6. Allam J, Kennedy C, Aksamit T, Dispenzieri A. Pulmonary manifestations in patients with POEMS syndrome: a retrospective review of 137 patients. Chest. 2008;133:969-74 pubmed publisher
    b>POEMS syndrome is a monoclonal plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes...
  7. Dispenzieri A. POEMS syndrome. Blood Rev. 2007;21:285-99 pubmed
    b>POEMS syndrome is a rare paraneoplastic syndrome secondary to a plasma cell dyscrasia...
  8. Scarlato M, Previtali S. POEMS syndrome: the matter-of-fact approach. Curr Opin Neurol. 2011;24:491-6 pubmed publisher
    ..The aim is to provide an up-to-date overview of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome with special regard to the available therapy options...
  9. 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;84:182-5 pubmed
    ..Our experience suggests that autologous purged stem cell transplantation should be considered as the treatment of choice for POEMS syndrome.

More Information

Publications62

  1. Ganti A, Pipinos I, Culcea E, Armitage J, Tarantolo S. Successful hematopoietic stem-cell transplantation in multicentric Castleman disease complicated by POEMS syndrome. Am J Hematol. 2005;79:206-10 pubmed
    ..A diagnosis of multicentric Castleman disease with POEMS syndrome was made, and he then received high-dose chemotherapy with melphalan followed by autologous peripheral blood ..
  2. Lewerenz J, Gocht A, Hoeger P, Von Den Driesch P, Eckert B, Lamszus K, et al. Multiple vascular abnormalities and a paradoxical combination of vitamin B12 deficiency and thrombocytosis in a case with POEMS syndrome. J Neurol. 2003;250:1488-91 pubmed
    ..We report a case of POEMS syndrome due to a gammopathy of undetermined significance with thrombocytosis, vitamin B(12) deficiency, highly ..
  3. Soubrier M, Ruivard M, Dubost J, Sauvezie B, Philippe P. Successful use of autologous bone marrow transplantation in treating a patient with POEMS syndrome. Bone Marrow Transplant. 2002;30:61-2 pubmed
  4. Rovira M, Carreras E, Blade J, Graus F, Valls J, Fernandez Aviles F, et al. Dramatic improvement of POEMS syndrome following autologous haematopoietic cell transplantation. Br J Haematol. 2001;115:373-5 pubmed
    b>POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, serum monoclonal protein and skin changes) is a rare plasma cell disorder of unknown pathogenesis and is diagnosed by the demonstration of a plasma cell proliferation at the ..
  5. Jaccard A, Royer B, Bordessoule D, Brouet J, Fermand J. High-dose therapy and autologous blood stem cell transplantation in POEMS syndrome. Blood. 2002;99:3057-9 pubmed
    ..HDT with stem cell support should be investigated further as a therapeutic option in patients with POEMS syndrome and disseminated plasma cell dyscrasia.
  6. Peggs K, Paneesha S, Kottaridis P, Chakraverty R, Tobias G, Reilly M, et al. Peripheral blood stem cell transplantation for POEMS syndrome. Bone Marrow Transplant. 2002;30:401-4 pubmed
    ..systemic AL amyloidosis) the possible benefits of dose intensification are yet to be fully explored in POEMS syndrome. One important issue is whether the toxicity of the procedure is significantly increased in this group...
  7. Watanabe O, Maruyama I, Arimura K, Kitajima I, Arimura H, Hanatani M, et al. Overproduction of vascular endothelial growth factor/vascular permeability factor is causative in Crow-Fukase (POEMS) syndrome. Muscle Nerve. 1998;21:1390-7 pubmed
    Crow-Fukase or POEMS syndrome of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes is a rare multisystem disorder of obscure pathogenesis that is associated with microangiopathy, neovascularization, and accelerated ..
  8. Ashawesh K, Prassad M, Murthy N, Fiad T. Polyneuropathy in POEMS syndrome. Eur J Haematol. 2008;81:403 pubmed
  9. Abrisqueta P, Rovira M, Cibeira M, Vallansot R, Gutiérrez G, Blade J, et al. [Autologous haematopoietic stem cell transplantation in POEMS syndrome: results in 4 cases]. Med Clin (Barc). 2007;129:578-81 pubmed
    ..A specific treatment for this disease does not exist. We report 4 patients with POEMS syndrome treated with an autologous haematopoietic stem cell transplantation in our centre...
  10. Mineta M, Hatori M, Sano H, Hosaka M, Kokubun S, Hiroki E, et al. Recurrent Crow-Fukase syndrome associated with increased serum levels of vascular endothelial growth factor: a case report and review of the literature. Tohoku J Exp Med. 2006;210:269-77 pubmed
    ..The serum level of VEGF well correlated to the clinical course of the patient. In conclusion, measurement of the VEGF level is useful for diagnosing CFS and for monitoring its clinical course...
  11. Phillips J, Dixon J, Richardson J, Fabre V, Callen J. Glomeruloid hemangioma leading to a diagnosis of POEMS syndrome. J Am Acad Dermatol. 2006;55:149-52 pubmed
    ..Of the skin changes, the glomeruloid hemangioma is considered to be a specific marker of POEMS syndrome. We describe a 68-year-old man who presented to his dermatologist with multiple hemangiomas, whose biopsy ..
  12. Kuwabara S, Misawa S, Kanai K, Kikkawa Y, Nishimura M, Nakaseko C, et al. Autologous peripheral blood stem cell transplantation for POEMS syndrome. Neurology. 2006;66:105-7 pubmed
    ..The authors treated four patients with high-dose chemotherapy and autologous peripheral blood stem cell transplantation. Within 6 months, symptoms associated with rapid normalization of serum VEGF levels improved...
  13. Badros A, Porter N, Zimrin A. Bevacizumab therapy for POEMS syndrome. Blood. 2005;106:1135 pubmed
  14. Wiesmann A, Weissert R, Kanz L, Einsele H. Long-term follow-up on a patient with incomplete POEMS syndrome undergoing high-dose therapy and autologous blood stem cell transplantation. Blood. 2002;100:2679-80 pubmed
  15. Lucchetta M, Pazzaglia C, Granata G, Briani C, Padua L. Ultrasound evaluation of peripheral neuropathy in POEMS syndrome. Muscle Nerve. 2011;44:868-72 pubmed publisher
    ..The aim of our study was to assess the pattern of ultrasound (US) nerve abnormalities in POEMS syndrome patients.
  16. Kuwabara S, Misawa S, Kanai K, Sawai S, Hattori T, Nishimura M, et al. Thalidomide reduces serum VEGF levels and improves peripheral neuropathy in POEMS syndrome. J Neurol Neurosurg Psychiatry. 2008;79:1255-7 pubmed publisher
  17. 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;47:672-9 pubmed
    b>POEMS syndrome is a rare plasma cell disorder, characterized by polyneuropathy, organomegaly, endocrinopathy, serum monoclonal protein, and skin lesions...
  18. Albertí M, Martinez Yelamos S, Fernandez A, Vidaller A, Narvaez J, Cano L, et al. 18F-FDG PET/CT in the evaluation of POEMS syndrome. Eur J Radiol. 2010;76:180-2 pubmed publisher
    In POEMS syndrome the identification and biopsy of an osteosclerotic lesion or a lymph node typical of Castleman's disease (CD) is essential to establish the diagnosis and plan appropriate treatment...
  19. Kastritis E, Terpos E, Anagnostopoulos A, Xilouri I, Dimopoulos M. 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;7:73-6 pubmed
    ..Decreasing VEGF levels parallel clinical improvement, and the restoration of normal bone metabolism follows HDT...
  20. Imai N, Kitamura E, Tachibana T, Konishi T, Suzuki Y, Serizawa M, et al. Efficacy of autologous peripheral blood stem cell transplantation in POEMS syndrome with polyneuropathy. Intern Med. 2007;46:135-8 pubmed
    b>POEMS syndrome (POEMS) is a rare multi-system disease which is typically associated with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes...
  21. Baba T, Østerby R, Neugebauer Baba S, Nozawa Y, Sakurai K, Katoh T, et al. No nephropathy in Type 2 diabetic patient with POEMS syndrome with an elevated plasma VEGF. Diabet Med. 2004;21:292-4 pubmed
    ..The findings obtained from this case did not support the hypothesis that VEGF may enhance the development of diabetic nephropathy...
  22. Velez D, Delgado Jimenez Y, Fraga J. Solitary glomeruloid haemangioma without POEMS syndrome. J Cutan Pathol. 2005;32:449-52 pubmed
    ..for a histologically distinctive cutaneous haemangioma, which they considered a specific cutaneous marker for POEMS syndrome. Glomeruloid haemangiomas appear to be specific to POEMS syndrome, because they have not been reported in ..
  23. Kuwabara S, Misawa S, Kanai K, Suzuki Y, Kikkawa Y, Sawai S, et al. Neurologic improvement after peripheral blood stem cell transplantation in POEMS syndrome. Neurology. 2008;71:1691-5 pubmed publisher
    ..There is increasing evidence that high-dose chemotherapy with autologous peripheral blood stem cell transplantation (Auto-PBSCT) is an efficacious treatment...
  24. Gütgemann I, Stevens K, Loftus D, Schmidt Wolf I, George T. VEGF and osteosclerosis in POEMS syndrome. Ann Hematol. 2008;87:243-5 pubmed publisher
  25. Kuwabara S, Dispenzieri A, Arimura K, Misawa S, Nakaseko C. Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome. Cochrane Database Syst Rev. 2012;:CD006828 pubmed publisher
    ..The pathogenesis of POEMS syndrome is not well understood, but overproduction of vascular endothelial growth factor (VEGF), probably secreted by ..
  26. Pina Oviedo S, López Patiño S, Ortiz Hidalgo C. Glomeruloid hemangiomas localized to the skin of the trunk with no clinical features of POEMS syndrome. Int J Dermatol. 2006;45:1449-50 pubmed
  27. Inoue D, Kato A, Tabata S, Kitai T, Takiuchi Y, Kimura T, et al. Successful treatment of POEMS syndrome complicated by severe congestive heart failure with thalidomide. Intern Med. 2010;49:461-6 pubmed
    A 55-year-old woman was diagnosed with POEMS syndrome; however, we could not perform high-dose chemotherapy with autologous peripheral blood stem cell transplantation (auto-PBSCT) because of rapidly progressive congestive heart failure...
  28. De Roma I, Filotico R, Cea M, Procaccio P, Perosa F. Calciphylaxis in a patient with POEMS syndrome without renal failure and/or hyperparathyroidism. A case report. Ann Ital Med Int. 2004;19:283-7 pubmed
    ..Two and a half years earlier, a POEMS syndrome had been diagnosed on the basis of a history of organomegaly and mild lymphadenopathy, IgA-lambda monoclonal ..
  29. Sinisalo M, Hietaharju A, Sauranen J, Wirta O. Thalidomide in POEMS syndrome: case report. Am J Hematol. 2004;76:66-8 pubmed
    We report a patient with incapacitating POEMS syndrome characterized by serum monoclonal protein, polyneuropathy, organomegaly, endocrinopathy, mesangiocapillary glomerulonephritis, massive ascites formation, and pulmonary hypertension...
  30. Hogan W, Lacy M, Wiseman G, Fealey R, Dispenzieri A, Gertz M. Successful treatment of POEMS syndrome with autologous hematopoietic progenitor cell transplantation. Bone Marrow Transplant. 2001;28:305-9 pubmed
    b>POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a plasma cell dyscrasia that differs substantially from classic multiple myeloma...
  31. Singh D, Wadhwa J, Kumar L, Raina V, Agarwal A, Kochupillai V. POEMS syndrome: experience with fourteen cases. Leuk Lymphoma. 2003;44:1749-52 pubmed
    The POEMS syndrome, also known as Crow-Fukase disease, is a rare multisystem disorder, which may take several years to evolve fully...
  32. Chan P, Lee K, Chong L, Lo K, Cheung Y. Glomeruloid haemangioma with cerebriform morphology in a patient with POEMS syndrome. Clin Exp Dermatol. 2006;31:775-7 pubmed
    ..After chemotherapy, other manifestations of POEMS syndrome developed, such as multiple haemangiomas over the chest and neck region, splenomegaly and generalized oedema...
  33. Kotani Y, Ito M, Abumi K, Yasui K, Minami A. Neurological recovery from pentaplegia after resection for sacral plasmacytoma presenting with POEMS syndrome. J Neurosurg Spine. 2009;10:324-8 pubmed publisher
    ..a case of a 52-year-old woman in whom tetraplegia developed with neurological respiratory failure due to POEMS syndrome associated with a solitary sacral plasmacytoma...
  34. Chee C, Dispenzieri A, Gertz M. Amyloidosis and POEMS syndrome. Expert Opin Pharmacother. 2010;11:1501-14 pubmed publisher
  35. D Souza A, Lacy M, Gertz M, Kumar S, Buadi F, Hayman S, et al. Long-term outcomes after autologous stem cell transplantation for patients with POEMS syndrome (osteosclerotic myeloma): a single-center experience. Blood. 2012;120:56-62 pubmed publisher
    The POEMS syndrome (polyradiculoneuropathy, organomegaly, multiple endocrinopathies, monoclonal protein, skin changes) is a rare disease associated with a plasma cell dyscrasia...
  36. Scarlato M, Previtali S, Carpo M, Pareyson D, Briani C, Del Bo R, et al. Polyneuropathy in POEMS syndrome: role of angiogenic factors in the pathogenesis. Brain. 2005;128:1911-20 pubmed
    ..We found that POEMS syndrome was associated with high levels of serum VEGF and, conversely, low levels of serum EPO...
  37. Soubrier M, Sauron C, Souweine B, Larroche C, Wechsler B, Guillevin L, et al. Growth factors and proinflammatory cytokines in the renal involvement of POEMS syndrome. Am J Kidney Dis. 1999;34:633-8 pubmed
    The POEMS syndrome is a multisystemic syndrome associated with plasma cell dyscrasia, characterized by the combination of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes...
  38. Forman S, Tyler W, Ferringer T, Elston D. Glomeruloid hemangiomas without POEMS syndrome: series of three cases. J Cutan Pathol. 2007;34:956-7 pubmed
  39. Jouve P, Humbert M, Chauveheid M, Jais X, Papo T. POEMS syndrome-related pulmonary hypertension is steroid-responsive. Respir Med. 2007;101:353-5 pubmed
    ..The POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal immunoglobulin, Skin changes) syndrome is a rare disease that entails a specific risk for pulmonary arterial hypertension...
  40. D Souza A, Hayman S, Buadi F, Mauermann M, Lacy M, Gertz M, et al. The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome. Blood. 2011;118:4663-5 pubmed publisher
    The POEMS syndrome is associated with elevated vascular endothelial growth factor (VEGF) levels...
  41. Dispenzieri A, Moreno Aspitia A, Suarez G, Lacy M, 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. 2004;104:3400-7 pubmed
    b>POEMS syndrome is characterized by peripheral neuropathy (PN), a clonal plasma cell disorder (PCD), organomegaly, endocrinopathy, skin changes, edema, sclerotic bone lesions, and thrombocytosis...
  42. Li J, Zhou D, Huang Z, Jiao L, Duan M, Zhang W, et al. Clinical characteristics and long-term outcome of patients with POEMS syndrome in China. Ann Hematol. 2011;90:819-26 pubmed publisher
    b>POEMS syndrome is a rare plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes...
  43. Kawano Y, Nakama T, Hata H, Kimura E, Maruyoshi N, Uchino M, et al. Successful treatment with rituximab and thalidomide of POEMS syndrome associated with Waldenstrom macroglobulinemia. J Neurol Sci. 2010;297:101-4 pubmed publisher
    A POEMS syndrome is a rare disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin abnormalities including hyperpigmentation and hypertrichosis...
  44. Humeniuk M, Gertz M, Lacy M, Kyle R, Witzig T, Kumar S, et al. Outcomes of patients with POEMS syndrome treated initially with radiation. Blood. 2013;122:68-73 pubmed publisher
    b>POEMS syndrome is a rare plasma cell dyscrasia presenting with polyneuropathy and other systemic findings...
  45. Isose S, Misawa S, Kanai K, Shibuya K, Sekiguchi Y, Nasu S, et al. POEMS syndrome with Guillan-Barre syndrome-like acute onset: a case report and review of neurological progression in 30 cases. J Neurol Neurosurg Psychiatry. 2011;82:678-80 pubmed publisher
    ..Herein, the authors report a 34-year-old woman with POEMS syndrome presenting as acute polyneuropathy...
  46. Rached S, Athanazio R, Dias S, Jardim C, Souza R. Systemic corticosteroids as first-line treatment in pulmonary hypertension associated with POEMS syndrome. J Bras Pneumol. 2009;35:804-8 pubmed
    The POEMS syndrome is a rare plasma cell disease...
  47. Dispenzieri A, Klein C, Mauermann M. Lenalidomide therapy in a patient with POEMS syndrome. Blood. 2007;110:1075-6 pubmed
  48. Kim S, Lee S, Ryoo H, Lee K, Hyun M, Bae S. Thalidomide for POEMS syndrome. Ann Hematol. 2006;85:545-6 pubmed
  49. Dispenzieri A, Gertz M. Treatment of POEMS syndrome. Curr Treat Options Oncol. 2004;5:249-57 pubmed
    ..Until the pathogenesis is fully understood, these are the mainstays of treatment for patients with POEMS syndrome.
  50. Hudnall S, Chen T, Brown K, Angel T, Schwartz M, Tyring S. Human herpesvirus-8-positive microvenular hemangioma in POEMS syndrome. Arch Pathol Lab Med. 2003;127:1034-6 pubmed
  51. Michizono K, Umehara F, Hashiguchi T, Arimura K, Matsuura E, Watanabe O, et al. Circulating levels of MMP-1, -2, -3, -9, and TIMP-1 are increased in POEMS syndrome. Neurology. 2001;56:807-10 pubmed
    ..inhibitor of metalloproteinases (TIMP), and vascular endothelial growth factor (VEGF) in blood samples of POEMS syndrome. Circulating levels of MMP-1, -2, -3, -9, and TIMP-1 were more increased in patients with POEMS syndrome than ..
  52. Wong V, Wade N. POEMS syndrome: an unusual cause of bilateral optic disk swelling. Am J Ophthalmol. 1998;126:452-4 pubmed
    To alert ophthalmologists to POEMS syndrome as an unusual cause of optic disk swelling.
  53. González Guerra E, Haro M, Farina M, Martin L, Manzarbeitia L, Requena L. Glomeruloid haemangioma is not always associated with POEMS syndrome. Clin Exp Dermatol. 2009;34:800-3 pubmed publisher
    ..To our knowledge, only two cases of glomeruloid haemangioma have been previously reported in patients without POEMS syndrome. We describe the third case of glomeruloid haemangioma in a patient without features of POEMS syndrome.