Complications of iron accumulation in patients with major thalassemia

A Banihashem MD, Associate Porf. Of pediatrics, Mashhad University of Medical Sciences.

S Hiradfar MD, Anatomoclinical pathologist.

R Gasemi MD.

Introduction: Thalassemia is the most common hereditary hemolytic disease in the Unfortunately Iran is located on the geographic distribution line of thalassemia. There are approximately 20000 cases of major thalassemia in the country. Most cases are in the north east and central areas.

Purpos: As thalassemia is common in Iran and need for multiple transfusions with it s attendant complications in these patients, we decided to evaluate if the standard dose and administration route of desferal is sufficient for prevention of complications of iron and accumulation.

Patients and method: Sorum ferritin, BS,ALT,AST,ALKph,T3,T4,GH assay was done in eighty patients (F:36,M:44) with at least 5 years of transfusion history . there was consanguineous marriage of parents in 65% of cases. Splenectomy had been done in 55% of cases . Desferal pump was used by 89% of patients.

Results: Serum ferritin:2% normal, 98% increased. Blood suger, ALT,AST, and ALKph were above normal value in 9% , 65%, 61% and 9% respectively . T3:96% normal,4% decreased.T4:98% normal 1% decreased 1% decreased GH:80%normal,3% decreased, 17% increased.

Conclusion: As serum ferritin was elevated in 98% of patients inspite of desferal pump use and there were cases of elevated blood sugar and impaired LFT,it seems prudent to reevaluate the current the current protocol of desferal administration