[Long term evolution in Blackfan-Diamond hypoplastic anemia (author's transl)].
Six cases of Blackfan-Diamond hypoplastic anemia are reported. Five were initiated within six months of life. All cases showed intense anemia, severe reticulopenia and hypersideremia. Two of them had associated malformations. Treatment was established with Dexametasone at high doses, followed by a maintenance treatment at lower doses. A partial response was obtained in all cases. Treatment with deforroxiamine was used in order to paliate hemosiderosis. Two cases developped microangiopathic hemolitic anemia; relations with primary disease could not be determined.
D000015:Abnormalities, Multiple; D000741:Anemia, Aplastic / Q000188:drug therapy; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004913:Erythrocytes, Abnormal; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D013577:Syndrome
Evolución a largo plazo en la anemia hipoplásica tipo Blackfan-Diamond