Author/Authors :
AI-Jaouni, Soad Khalil King abdulaiziz university - Faculty of medicine - Department of hematology, Saudi Arabia
Abstract :
Hemoglobin disorders are the most widespread, clinically serious, single-gene disorder in the world with an estimated 60,000-70,000 as beta thalassemia major. Its occurrence is seen at a high gene frequency throughout the Mediterranean and Middle Eastern populations. The standard treatment for thalassemia major has been transfusion-dependent and ironchelating therapy.The prognosis of patients with thalassemia major has been improved by transfusion and ironchelation therapy. Iron accumulation is fatal in the absence or inadequate chelating therapy.Hemoglobin disorders are the most widespread, clinically serious, single-gene disorder in the world with an estimated 60,000-70,000 as beta thalassemia major. Its occurrence is seen at a high gene frequency throughout the Mediterranean and Middle Eastern populations. The standard treatment for thalassemia major has been transfusion-dependent and ironchelating therapy.The prognosis of patients with thalassemia major has been improved by transfusion and ironchelation therapy. Iron accumulation is fatal in the absence or inadequate chelating therapy.regularly used worldwide as iron in vivo measurement and reflects the indirect way of measuring body iron stores. Serumferritin decreases by ascorbate deficiency and increases by infection, inflammation, liver dysfunction, and ineffective erythropoiesis. In spite serum ferritin is not precise in estimates of body iron burden, but still a common practice to monitor iron overload. Iron toxicity is the crucial factor for tissue damage observed in iron overloaded patients and makes effective chelation an absolute requirement to alleviate its impact. Accurate assessment of body iron is essential, especially in the major cause of death among thalassemic patients, e.g., cardiac diseases.