It is a chronic congenital hemolytic anemia in which defect seems to be inability to produce normal Hb.
Types of thalassemia:
- Thalassemia major: it is transmitted when both parents have thalassemia minor. Such children will have severe progressive anemia not compatible with normal life. The condition is recognized in early infancy because of the onset of progressive severe anemia,splenomegaly.
- Thalassemia intermediate: delay onset, less blood transfusion is required.
- Thalassemia minor(trait) or carrier: shows mild anemia, sometimes the spleen is enlarged, they live normal life, and they require no treatment.
Diagnosis of thalassemia:
C.B.P+Hb electrophoresis which is the diagnostic test.
Treatment of thalassemia:
- Frequent blood transfusion to maintain the Hb level above 10 gram/dl.
- Splenectomy may be helpful. It lessen the discomfort from enlarged spleen, it also help to prolong the interval between blood transfusion.
- Desferal administration subcutaneously daily for 5 days every week by infusion pump over 8-12 hours.
- Vitamin C given orally at the same time of desferal injection.
- Folic acid is necessary for every chronic hemolytic anemia.
- Bone marrow transplantation .
- Gene therapy in the recent future.