García-Miguel P, Muñoz Villa A, Hurtado Ruano T, González Martín M C, Fernández Rodríguez P
An Esp Pediatr. 1976 Jul-Aug;9(4):384-90.
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.
报告了6例黑范-戴蒙德发育不全性贫血。其中5例在出生后6个月内发病。所有病例均表现为严重贫血、重度网织红细胞减少和高铁血症。其中2例伴有畸形。采用大剂量地塞米松进行治疗，随后以较低剂量维持治疗。所有病例均获得部分缓解。使用去铁胺进行治疗以缓解含铁血黄素沉着症。2例发生微血管病性溶血性贫血；与原发性疾病的关系无法确定。
