摘要
目的 :探讨重型 β地中海贫血 (重型 β地贫 )并发急性免疫性溶血的临床特点、治疗和预后。 方法 :10例患儿是 1996年 1月～ 2 0 0 4年 3月本院住院患者 ,男 7例 ,女 3例 ,年龄 9个月～ 15岁 ,确诊重型 β地贫 1个月～ 11年 ,均依靠输血治疗 ,原Hb维持在 70～ 14 0g/L。入院时患儿表现为慢性溶血基础上于感染或输血后发生急性溶血 ,2周内Hb平均下降 4 5 g/L ,伴黄疸、血尿 ,间接胆红素、血游离Hb明显升高等血管内溶血表现。 10例直接Coomb试验阳性 ,其中 7例伴C3 抗体阳性 ;血清抗IgG均阴性 ;1例患者Rh血型为CCDee,输ABO同型血后发病 ,血清特异性抗cE抗体阳性 ;9例直接Coomb试验阳性病例血清无血型免疫性不规则抗体 ;1例在配血时发现自身血液有凝集现象 ,检查Coomb试验阳性。确诊后均予激素治疗 ,7例予甲泼尼龙 2 0～ 30mg/kg·d -1× (3～ 4 )d ;1例予地塞米松 0 .5mg/kg·d-1× 3d ;2例治疗之初即予泼尼松口服 2mg/kg·d-1;急性溶血控制后均予口服泼尼松维持治疗 4周以上 ;3例需加用CsA ,1例加用骁悉 ,3例同时使用丙种球蛋白 (IVIG) 2g/kg。输注经严格交叉配血 (患儿血与献血者血在生理盐水、酶、抗人球蛋白介质中交叉配血无凝血、无溶血现象 )的洗涤浓缩红细胞 35ml/kg 。
Objective:To observe the clinical charateristics, treatment and prognosis of beta-thalassemia major patients complicated by immune-mediated hemolytic anemia.Method:Ten patients with beta-thalassemia major started on regular transfusions to maitain haemoglobin(Hb)level between 70～140 g/L. After infection (9 cases) and transfusions (1 case), acute haemolysis developed suddenly with jaundice, haemoglubinuria and Hb declined dramaticly within 2 weeks. The Coombs test was positive, with presence of autoantibodies anti-C 3 in 7 cases and alloantibodies anti-E and anti-c in 1 patients (the patient Rh antigens being CCDee). Immunosuppressive treatment with corticosteroid were immediately started and all changed to maintaining treatment with prednisone after controlling the acute immune-mediated haemolysis. One patient needed combination of corticosteroid and cyclosporine (CsA), another patient needed combination of corticosteroid and mycophenolate mofetil (MMF).Result:All patients were followed up for 3 months to 8 years. 8 patients obtained coombs test negative 2 to 8 weeks later. Their seral antibodies level remains undectable. They accepted prednisone for 4 weeks to 6 months and none of them relapsed after drug withdrawal. 2 patients got recurrent infections, their pathogenetic condition aggravated repeatedly. One of them received maintence therapy with prednisone and CsA for 1 year, while the other gradually obtained anti-C 3 antibody negative after usage with prednisone and MMF for 9 months, Moreover his condition stablized after administration of prednisone for 2 years.Conclusion:Beta-thalassemia patients complicated by immune-mediated hemolytic anemia can be treated by corticosteroid effectively. Corticosteroid should not be reduced until Coombs test changed to negative.
出处
《临床血液学杂志》
CAS
2005年第1期3-6,共4页
Journal of Clinical Hematology
