Chelation

Chelation is necessary because of the regular blood transfusions?  Or is there any other reason why the iron in the blood builds up?

Iron buildup is caused by two things. The first is transfusion. The body has no mechanism for removing excess iron so it builds with each transfusion. Second, when the hemoglobin levels are low, iron absorption from the diet increases. In non-transfused thal intermedias it can cause a significant problem as the absorption rate can be as high as 100 times normal, resulting in dangerous iron overload conditions, requiring chelation. In majors, iron absorption is related to how low the Hb is allowed to drop between transfusions. The lower the Hb, the higher iron absorption is in the gut.

http://www.chiangmairam.com/Clinic_new/Talas2.jpg

What is the cause of this deformatity?

H i emersive,

the child shows typical signs of hepato-splenomegaly caused by exsessive ineffective erytropoiesis and a lot of factors in Thals. Splen and liver try to filtering out old bad and damaged red blood cells. Unfortunately thals are producing a lot of defected RBC's, so the splen have a lot of work to do and enlarge littel by littel. Unwell treated thals show these enlargement more often than well treated thal with regular transfusion or an Hb around 9 g/dl. If the enlargement is to big splenectomy is the solution to give an chance to survive.