[Thalassaemia intermedia. Clinical and laboratory study. Therapeutic suggestions (author's transl)].
The clinical and laboratory criteria which distinguish thalassaemia intermedia (T.I.) from thalassaemia major were analyzed in a series of 30 patients with homozygous beta- thalassaemia, 8 of whom had T.I. The appearance of the first symptoms after the age of 2 years, the moderate spleen enlargement, the haemoglobin levels approaching 8 g/100 ml and the response to moderate transfusions over 1-year observation period were in favour of T.I. Since patients who had transfusions were clinically better than those who had none, it is suggested that T.I. patients should be treated with regular transfusions and iron chelating agents.
D000367:Age Factors; D001803:Blood Transfusion; D002648:Child; D002675:Child, Preschool; D003676:Deferoxamine / Q000627:therapeutic use; D005260:Female; D005319:Fetal Hemoglobin / Q000032:analysis; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D013163:Splenomegaly / Q000209:etiology; D013789:Thalassemia / Q000097:blood / Q000175:diagnosis* / Q000628:therapy
D006454:Hemoglobins; D005319:Fetal Hemoglobin; D003676:Deferoxamine
La thalassémie intermédiaire. Etude clinique et biologique. Propositions thérapeutiques.