ISSN: 1300-0292
İndekslendiği Dizinler: SCIENCE CITATION INDEX EXPANDED
CINAHL, Index Copernicus,
Chemical Abstracts (CA),
Excerpta Medica / EMBASE
Dil: Türkçe, İngilizce
İçerik: Orijinal Araştırma, Derleme, Editöre Mektup, Olgu Sunumu, Tıp Eğitimi, Tıbbi Kitap İncelemeleri
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Sideroblastic Anemias
Cengiz BEYAN*, Kürşad KAPTAN**, Türker ÇETİN***
* Doç.Dr., Gülhane Askeri Tıp Akademisi Hematoloji BD,
** Uz.Dr., Gülhane Askeri Tıp Akademisi Hematoloji BD,
*** Yrd.Doç.Dr., Gülhane Askeri Tıp Akademisi Hematoloji BD, ANKARA
Sideroblastic anemias occur in both inherited and acquired forms usually due to a defect of heme biosynthesis in erythroid cells. Inherited X-linked sideroblastic anaemia has been shown to be caused by mutations in the erythroid-specific ALA synthase gene. Generally the acquired form is a disease of older adults and is considered a myelodysplastic syndrome. Sideroblastic anemias are often accompanied by hypochromia, and microcytosis in inherited form, macrocytosis of erythrocytes in acquired form. This group of disorders is characterized by the presence of ringed sideroblasts in the bone marrow and increased stainable iron in bone marrow macrophages. Many patients with inherited sideroblastic anemia remain stable for years with a relatively mild degree of anemia and require no specific treatment. Both the inherited and acquired forms are characterized by markedly increased ineffective erythropoiesis, which is associated with increased intestinal iron absorption. Such patients may develop iron overload that is a major complication of this disorder. Some patients with anemia may respond to pharmacological doses of pyridoxine, but blood transfusions become necessary in the majority of these patients.Keywords: Sideroblastic AnemiaTurkiye Klinikleri J Med Sci 2001, 21:432-437
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