It is understood that sideroblastic anemia is the result of defects in the steps of heme biosynthesis that occur within the mitochondrion. Sideroblasts are the pathognomic feature of sideroblastic anemia. There is deffect in incorporation of iron in to heme. As a result the iron accumulates in mitochondria of red cell precursors. Ring sideroblasts are erythroblasts that have iron-loaded mitochondria. The iron granules are arranged around the nucleus in a ring form. They can be seen with prussian blue staining as blue granules around the nucleus.The pathophysiology of sideroblastic anemia depends on the underlying cause. Impaired hemoglobin production, results in reduced number of mature erythrocytes. Resulting anemia is usually microcytic and hypochromic. The iron overload in sideroblastic anemia is due to  abnormalities in iron utilization. There is increased iron transport to erythroblasts. Since the body sense anemia intestinal iron absorption increases. There is increased iron content in mitochondria of erythroblasts and systemic iron accumulation. Systemic iron overload occurs only in some forms of sideroblastic anemia, usually when the defects in iron metabolisms involve earlier stages of erythroid pathways. The development of heriditory sideroblastic anemia is the result of multiple genetic mutations in several genes involved in heme synthesis resulting in autosomal recessive congenital sideroblastic anemia. Out of many genes SLC25A38 mutations is the most common.
Sideroblastic anemia pathophysiology
Iron deficiency anemia pathophysiology
Hemolytic anemia pathophysiology
Macrocytic anemia pathophysiology
Sideroblastic anemia risk factors
Microangiopathic hemolytic anemia causes
Macrocytic anemia risk factors
Hemolytic anemia risk factors