Long term evolution in Blackfan-Diamond hypoplastic anemia
García-Miguel, P.; Muñoz Villa, A.; Hurtado Ruano, T.; González Martín, M.C.; Fernández Rodríguez, P.
Anales Espanoles de Pediatria 9(4): 384-390
1976
ISSN/ISBN: 0302-4342 PMID: 984600 Document Number: 101066
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.