Poems syndrome in A-10-year-old girl: A case report

POEMS sendromu, plazma hücre diskrazisi ile ilişkili olarak polinöropati, organomegali, endokrinopati, M protein ve deri değişikliklerinden oluşan bir sendrom olarak tanımlanmaktadır. Bu yayında, polinöropati, kardiyomegali, hepatomegali, tip 1 diabetes mellitus, çift monoklonal gammapati (IgA ve IgG), hiperpigmentasyon ve hipertrikozisi olan 10 yaşındaki bir kız olguda POEMS sendromunu tanımlanmalıdır. Bilgilerimize göre yayımlanmış olan en genç olgudur.

Poems sendromu 10 yaşında kız olgu

POEMS syndrome has been defined as an association of plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes. In this report, we describe a case of POEMS syndrome. A 10-year-old girl presented with polyneoropathy, diabetes mellitus type I, double monoclonal gammopathy ( IgA and IgG ), hepatomegaly, cardiomegaly, hyperpigmentation, and hypertricosis. To our knowledge, she is the youngest case of POEMS syndrome who has been reported.

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  • 1. Nakanishi T, Sobue I, Toyokura Y, et al. The Crow-Fukase syndrome : a study of 102 cases in Japan . Neurology 1984; 34: 712- 720.
  • 2. Viard JP, Lesavre P, Boitard C, et al. POEMS syndrome presenting as systemic sclerosis: clinical and pathologic study of case with microanjiopathic glomerular lesions. Am J Med 1988; 84: 524-528.
  • 3. Soubrier MJ, Dubost JJ, Sauvezie JM, et al. POEMS syndrome: a study of 25 cases and a review of the litareture; French Study Group on POEMS Syndrome. Am J Med 1994; 97: 543-553.
  • 4. Bandwick PA, Zwaifler NJ, Gill GN, Newman D, Greenway GD: Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes: The POEMS syndrome. Medicine1980, 59: 311.
  • 5. Miralles GD, O' Fallon JR, Talley NJ: Plasma cell dyscrasia with polyneuropathy. The spectrum of POEMS syndrome. N Engl J Med 1992, 327:1919.
  • 6. Thomas B, Arthur Z, Klaus W, Irwin M, Frank A: POEMS syndrome. Dermatology in General Medicine, Volume I. McGraw-Hill, Inc. 1993, 1229-30.
  • 7. Gherardi R, Zuber M, Viard JP: Les neuropathies disglobulinemiques. Rev Neurol (Paris) 1988, 144: 391.
  • 8. Semble E, Challa VR, Hott DA, Pisko EJ. Light and electron microscopic findings in POEMS, or Japanese multisystem syndrome. Arthritis Rheum 1986; 29: 286-291.
  • 9. Sharief MK, McLean B, Thomson EJ: Elevated serum levels of tumor necrosis factor-a in Guillain-Barre syndrome. Ann Neurol 1991,333:591.
  • 10. Creange A, Belec L, Clair B, Raphael JC, Gherardi RK: Differential kinetics of circulating tumor necrosis factor (TNF)- alpha and soluble TNF reseptors (sTNFrs) in patients with Guillain-Barre syndrome (GBS). Neurology 1995,45: a 415, (suppl 4).
  • 11. Patton JS, Peters PM, McCabe J et al: Development of partial tolerance to the gastrointestinal effects of high doses of recombinant tumor necrosis factor-a in rodents. J Clin Invest 1987, 80:1587.
  • 12. Babak M, Manu J: Pulmonary manifestation of POEMS syndrome. CHEST 1999,115:1740-1742.
  • 13. Watanabe O, Arimura K, Kitajima I et ai. Greatly raised vascular endothelial growth factor (VEGF) in POEMS syndrome. Lancet 1996,347:702.
  • 14. Masson C, Krespi Y: POEMS syndrome. Ann Neurol 1994, 35: 501-505 .
  • 15. François-Jerome A, Laurent B, Yves Let al: Am Coll of Rheum. 1996, 39:1423-1426.
  • 16. Zanone T, Bastion Y, Saies G, Rieux C, Morel D et al. POEMS syndrome, arterial thrombosis and thrombocytosis. J Intern Med 1996, 240 (2): 107-9.
Ege Tıp Dergisi-Cover
  • ISSN: 1016-9113
  • Yayın Aralığı: Yılda 4 Sayı
  • Başlangıç: 1962
  • Yayıncı: Ersin HACIOĞLU