Sumoza Abraham, de Bisotti Renate, Sumoza David, Fairbanks Virgil
Hospital Central Enrique Tejera, Section of Hematology and Bone Marrow Transplantation, Universidad de Carabobo, Apartado Postal 917, Valencia-Carabobo, Venezuela.
Am J Hematol. 2002 Nov;71(3):161-5. doi: 10.1002/ajh.10205.
Cerebrovascular accident (CVA) is a major cause of morbidity and death in sickle cell anemia (SCA). Transfusion of packed erythrocytes is widely used to prevent this complication. However, chronic transfusion may lead to iron overload, alloimmunization, or infections. Cost and compliance may compromise transfusion therapy. A possible alternative, the prophylactic use of hydroxyurea (HU), has not been tried to determine whether it may prevent recurrent stroke. We used HU in five children with SCA who had suffered stroke, in three of them after a first episode and in the other two after a second CVA. Four had infarctive stroke and one a transient ischemic attack (TIA). Four patients took HU at a dose of 40 mg/kg/d, one patient at 30 mg/kg/d. None of the patients had recurrent stroke during 42-112 months of observation. None experienced pain crises. In all, HbF increased significantly and was maintained above 14.7% during treatment. The total Hb concentration increased 19.5 g/L (median) above the value before treatment. HU was well tolerated. None of the five children had leukopenia or thrombocytopenia during therapy. HU appears to prevent recurrence of stroke in SCA without risk of major toxicity.
脑血管意外（CVA）是镰状细胞贫血（SCA）发病和死亡的主要原因。输注浓缩红细胞被广泛用于预防这种并发症。然而，长期输血可能导致铁过载、同种免疫或感染。成本和依从性可能会影响输血治疗。一种可能的替代方法，即预防性使用羟基脲（HU），尚未尝试确定其是否可以预防复发性中风。我们对5例患有SCA且发生过中风的儿童使用了HU，其中3例是首次发作后，另外2例是第二次CVA后。4例为梗死性中风，1例为短暂性脑缺血发作（TIA）。4例患者服用HU的剂量为40mg/kg/d，1例患者为30mg/kg/d。在42至112个月的观察期内，所有患者均未发生复发性中风。无一例经历疼痛危象。总体而言，HbF显著增加，且在治疗期间维持在14.7%以上。总血红蛋白浓度比治疗前的值增加了19.5g/L（中位数）。HU耐受性良好。5名儿童在治疗期间均未出现白细胞减少或血小板减少。HU似乎可以预防SCA中风复发，且无重大毒性风险。
