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Hb H HASTALIĞI
Results for "Hb H HASTALIĞI"
Hb H HASTALIĞI
(Medicine and Hematology Glossary) :
Hemoglobindeki a globulin sentezinde azalma ile seyreden bir hastalık. Bu hastalarda normalde 4 adet olması gereken alfa globulin genlerinden üçü eksiktir. Çok az miktarda HbA yapılabilir. Alfa globulin eksik olduğu için beta globulin zincirlerinde birikim olur ve HbH (b4 ) olarak bilinen anormal hemoglobin oluşur. Bu anormal hemoglobin unstabil davranış gösterir, yani oksidatif stres karşısında (enfeksiyon, oksidan ilaçlar) eritrositler yıkılır. Anemi diğer b thalassemilerden daha hafiftir. Hastalarda genellikle 7-10 gr/dL düzeyinde anemi, orta düzeyde splenomegali vardır. Edinsel Hb H hastalığı myeloproliferatif ve myelodisplastik bozukluklar sonucu gelişebilir.
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