POEMS syndrome: A case report

Amaç & Hipotez:43 yaşındaki kadın hasta 2,5 yıldır devam eden alt ekstremitelerde simetrik sensorimotor polinöropati, hipertrikozis, terleme, ishal, kilo kaybı ve pigmentasyon artışı ile servisimize kabul edildi.

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Temel Bulgular & Veri:Klinik değerlendirmede bulgular POEMS Sendromu’nun tanı kriterleri ile uyumluydu.

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Sonuç & Katkı:Burada 2 gram/gün proteinürisi ve hipertrofik osteoartropatisi ile diğer olgulardan farklı bir olgu sunduk.

🔬Tür:Deneysel Tarla Denemesi

📍Kapsam:Genişletilmiş Akademik Örneklem

⚙️Değişkenler:Siklofosfamid · İlaç tedavisi · kombinasyon

Özet

43 yaşındaki kadın hasta 2,5 yıldır devam eden alt ekstremitelerde simetrik sensorimotor polinöropati, hipertrikozis, terleme, ishal, kilo kaybı ve pigmentasyon artışı ile servisimize kabul edildi. Klinik değerlendirmede bulgular POEMS Sendromu’nun tanı kriterleri ile uyumluydu. Hasta başlangıçta prednizolon ile tedavi edildi ve iyi yanıt alındı. Biz otolog yüksek doz kemoterapi sonrası otolog kök hücre nakli planladık. Burada 2 gram/gün proteinürisi ve hipertrofik osteoartropatisi ile diğer olgulardan farklı bir olgu sunduk.

Abstract

A 43-year-old female patient admitted with a 2.5-year history of lower extremity symmetrical sensorimotor polyneuropathy, hypertrichosis, sweating, diarrhea, weight loss, and hyperpigmentation. The clinical evaluation met the criteria for the diagnosis of POEMS syndrome. The patient was initially treated with prednisolone and responded well. We planned high-dose chemotherapy with autologous stem cell rescue and introduced a more immunosuppressive regimen containing cyclophosphamide and dexamethasone. We present a case differing from the other cases with her 2 g/day proteinuria and hypertrophic osteoarthropathy.

Anahtar kelimeler

Siklofosfamid İlaç tedavisi kombinasyon Glukokortikoidler İmmün baskılayıcı ajanlar POEMS sendromu Kadın Erişkin Deksametazon Tanı ayırıcı Cyclophosphamide Drug Therapy Combination Glucocorticoids Immunosuppressive Agents POEMS Syndrome Female Adult Dexamethasone Diagnosis Differential

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Bu Makaleye Atıf Yap

ÖZDEN, A., SÜLEYMAN, Z., SEVEN, G., ARAT, M., AKIN, A., ŞENER, Ö., KUZU, I., KEVEN, K., ENSARİ, A., ERDEN, A., TAN, E. (2008). POEMS syndrome: A case report. Turkish Journal of Gastroenterology, Cilt: 19 - Sayı: 4, 276–280. https://ajindex.com/articles/409908

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Kaynakça (13)

Nakanishi T, Sobue I, Toyokura Y, et al. The Crow-Fukase syndrome: a study of 102 cases in Japan. Neurology 1984; 34:712-20
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 1994;97:543-53
Dispenzieri A, Kyle RA, Lacy MQ, et al. POEMS syndrome: definitions and long-term outcome. Blood 2003;101:2496- 506
Dispenzieri A. POEMS syndrome. Blood Rev 2007;21:285- 99
Orefice G, Morra VB, De Michele G, et al. POEMS syndrome: clinical, pathological and immunological study of a case. Neurol Res 1994;16:477-80
Watanabe O, Maruyama I, Arimura K, et al. Overproduction of vascular endothelial growth factor/vascular permeability factor is causative in Crow-Fukase (POEMS) syndrome. Muscle Nerve 1998;21:1390-7
Martínez-Lavín M. Hypertrophic osteoarthropathy. Curr Opin Rheumatol 1997;9: 836
Laurenti L, de Matteis S, Sabatelli M, et al. Early diagnosis followed by front-line autologous peripheral blood stem cell transplantation for patients affected by POEMS syndrome. Leuk Res 2008;32:1309-12. 280 ÖZDEN et al
Kanai K, Kuwabara S, Misawa S, Hattori T. Failure of treatment with anti-VEGF monoclonal antibody for long-standing POEMS syndrome. Intern Med 2007;46:311-3
Dispenzieri A, Moreno-Aspitia A, Suarez GA, et al. Peripheral blood stem cell transplantation in 16 patients with POEMS syndrome, and review of the literature. Blood 2004;104:3400-7
Ganti AK, Pipinos I, Culcea E, et al. Successful hematopoietic stem-cell transplantation in multicentric Castleman disease complicated by POEMS syndrome. Am J Hematol 2005;79:206-10
Imai N, Kitamura E, Tachibana T, et al. Efficacy of autologous peripheral blood stem cell transplantation in POEMS syndrome with polyneuropathy. Intern Med 2007;46:135-8
Sonada S, Ookawara S, Karube H, 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.