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MİYELODİSPLASTİK SENDROMDA DEMİR BİRİKİMİ VE TEDAVİSİ: OLGU SUNUMU

Journal Name:

  • İstanbul Tıp Fakültesi Dergisi

Publication Year:

  • 2002

Key Words:

Keywords (Original Language):

Author NameUniversity of AuthorFaculty of Author
Abstract (2. Language): 
Management ofthe iron overload in the myelodysplastic syndrome: A case report. Myelodys-plastic syndrome (MDS) is a clonal hematological disorder characterized by refractory cytope-nia and increased risk of transformation to acute leukemia. MDS ranges from relatively mild anemia, to that more severe three lineage disorder with rapid evolution to acute leukemia. Se-condary hemochromatosis caused by repeated red blood celi transfusions and ineffective eryt-hropoiesis is a majör problem in MDS patients with refractory anemia who had relatively long survival. Here we report a 65-year-old male patient with MDS who was diagnosed as refractory anemia and received recurrent red blood celi transfusions. He had developed iron overload in the liver, heart, and hypophysis. He received desferrioxamine therapy. He died with car-diac complications related with secondary hemochromatosis. The development of iron overload and the importance of treatment with desferrioxamine in patients with MDS were reviewed.
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Abstract (Original Language): 
Miyelodisplastik sendrom (MDS) periferik kanda refrakter sitopenilerin varlığı ve yüksek oranda akut lösemiye dönüşme riski ile karakterize, klonal bir hematolojik hastalıktır. MDS sadece anemi ile seyredebileceği gibi, kemik iliğinde üç seride de ağır displazi ile seyredip hızla akut lösemiye dönüşebilir. Lösemiye dönüşme riski düşük olan, nispeten uzun bir sağka-lım süresi olan refrakter anemili hastalarda en önemli komplikasyon, tekrarlayan kan transfüz-yonları ve inefektif eritropoez ile eritrosit transfüzyonları sonucu gelişen sekonder hemokro-matozdur. Bu yazıda refrakter anemi tanısıyla sürekli kan transfüzyonu yapılan, karaciğer, mi-yokard ve hipofizde demir birikimi gelişen, desferrioksamin ile şelasyon tedavisine rağmen demir birikimine bağlı kardiak komplikasyonlarla kaybedilen 65 yaşında bir erkek hasta sunuldu. Miyelodisplastik sendromda demir birikimi ve desferrioksamin ile şelasyon tedavisinin önemi tartışıldı.
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  • 4
273-278
  • Turkish

REFERENCES

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