Endocrinopathy in Hemochromatosis Patients Multi-Transfused for Aplastic Anemia / 대한소아혈액종양학회지

ABSTRACT

PURPOSE: Chronic blood transfusions result in excessive iron deposition leading to eventual tissue damage and impaired function of organs, such as the liver, spleen, pancreas, skin, thyroid, and heart. We evaluated the body iron status and endocrinopathy in repeatedly transfused patients with aplastic anemia (AA). METHODS: Fourteen patients with AA who were transfused with more than 10 Units of packed RBC since 1996 were evaluated. We evaluated the correlation of amount of blood transfused with status of iron stores (determined by serum iron, TIBC, ferritin and transferrin saturation) and organ damage. RESULTS: Patients received a median of 61 units (range 11~168 units) of PRC. Twelve patients (85.7%) had elevated serum ferritin levels, and 11 (78.6%) had elevated transferrin saturation. Serum ferritin (P<0.01; r=0.868), and transferrin saturation (P<0.05; r=0.569) were significantly correlated with the amount of PRC transfused, respectively. Five patients had clinically significant iron overload despite the use of deferoxamine. Organ damage caused by transfusion iron overload were skin pigmentation (N=3), hepatic (N=1) and endocrinologic abnormalities. Diabetes (N=3), hypothyroidism (N=3), and hyogonadotropic hypogonadism (N=1) were observed. No patient developed clinically significant arthropathy or cardiac disease. CONCLUSION: AA patients who received chronic blood transfusions develop iron overload which may lead to endocrinopathy. Iron status and organ dysfunction should be monitored and effective measures to prevent iron overload should be applied in patients who need chronic transfusions.