[Controlled desferrioxamine treatment of congenital anaemia and transfusion siderosis (author's transl)].
A 19-year-old patient with congenital Blackfan-Diamond anaemia has been maintained on a regular red cell transfusion schedule since he was two months old. From the age of four he has received desferrioxamine injections at regular intervals with different dose levels. In spite of the treatment with the chelating agent secondary siderosis developed with typical endocrinological abnormalities and cardiac arrhythmias. Increasing the dose of desferrioxamine to 16 g/24 h resulted in an iron excretion of 184 mg/24 h. Such an intensification of treatment finally produced a negative iron balance and the cardiac arrhythmias disappeared. Desferrioxamine therapy should be done under controlled circumstances only, an iron balance is mandatory, and the dose should be adjusted to the results of the balance.
D000293:Adolescent; D000741:Anemia, Aplastic / Q000188:drug therapy* / Q000628:therapy; D002648:Child; D003676:Deferoxamine / Q000008:administration & dosage / Q000627:therapeutic use*; D004913:Erythrocytes, Abnormal; D006801:Humans; D007501:Iron / Q000032:analysis; D008297:Male; D012806:Siderosis / Q000188:drug therapy* / Q000209:etiology; D013577:Syndrome; D065227:Transfusion Reaction
D007501:Iron; D003676:Deferoxamine
Kontrollierte Desferrioxamin-Therapie bei kongenitaler Anämie und Transfusionssiderose.