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本文报告了10例确诊的血红蛋白H 病行脾切除术治疗的效果,并根据血液学、临床表现的轻重程度分为三组:Ⅰ组为类似重型β地中海贫血组(4例);Ⅱ组为慢性溶血性黄疸组(2例):Ⅲ组为中度贫血组(4例)。三组作脾切除后均未作任何治疗,定期血液专科门诊追踪观察4个月—5年,术前重度贫血或脾亢者,术后血红蛋白增高明显,并以Ⅰ组疗效最佳,全部病例术后来发现如国外报告的反复严重感染,相反的感染频率较术前明显减少,也未发现切脾后的肝损害,因此,作者认为,凡年龄>3岁,Hb<7g/dl,尤其重度贫血伴巨脾的血红蛋白H 病儿,适于切脾治疗。
This article reports the results of 10 cases of confirmed hemoglobin H disease splenectomy treatment effect, and according to the severity of hematology, clinical manifestations were divided into three groups: Ⅰ group was similar to β-thalassemia major group (4 cases); Ⅱ group Chronic hemolytic jaundice group (2 cases): Ⅲ group was moderate anemia group (4 cases). Three groups of splenectomy were not for any treatment, regular blood specialist out-patient follow-up observation of 4 months to 5 years, preoperative severe anemia or hypersplenism, postoperative hemoglobin increased significantly, and the best effect in group Ⅰ, all cases After surgery, it was found that repeated serious infections such as foreign reports, the opposite frequency of infection was significantly reduced compared with preoperative, did not find liver damage after splenectomy, therefore, the authors believe that where age> 3 years old, Hb <7g / dl, Severe anemia with splenomegaly hemophilia H children, suitable for splenectomy.