Thalassemia Management

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Patients with thalassemia traits do not require medical or follow-up care after the initial diagnosis is made. Iron therapy should not be used unless a definite deficiency is confirmed and should be discontinued as soon as the potential Hb level for that individual is reached. Counseling is indicated in all persons with genetic disorders, especially when the family is at risk of a severe form of disease that may be prevented
Patients with severe thalassemia require medical treatment, and a blood transfusion regimen was the first measure effective in prolonging life,it was found to provide patients with many benefits, including reversal of the complications of anemia, elimination of ineffective erythropoiesis and its complications, allowance of normal or near-normal growth and development, and extension of patients' life spans. Blood transfusion should be initiated at an early age when the child is symptomatic and after an initial period of observation to assess whether the child can maintain an acceptable level of Hb without transfusion.
Accumulation of transfused iron and its consequences also needed to be addressed. Chelation therapy was considered after extensive research and many clinical trials. Today, regular blood transfusion combined with well-monitored chelation therapy has become the standard therapy and has drastically changed the outlook for this population of patients
Chelating agents . (Desferal) Deferoxamine mesylate
(Exjade ) Deferasirox
(Deferiprone) deferiperox
Folic acid deficiency : This deficiency is a common complication in patients with thalassemia, mainly because of the extreme demand associated with the severe expansion of the marrow. Other causes, such as poor absorption and intake, can also contribute to folate deficiency. For this reason, folic acid has been recommended as a supplement for this patient population
Hematopoietic stem cell transplantation (HCT) HSCT is recommended only for selected patients; it is the only known curative treatment for thalassemia. Poor outcome after HSCT correlates with the presence of hepatomegaly and portal fibrosis and with ineffective chelation prior to transplant. The event-free survival rate for patients who have all 3 features is 59%, compared to 90% for those who lack all 3.

Thalassemia Management

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What is Thalassemia