地中海贫血综合征的手术治疗

Surgical Treatment of Thalassemia Syndrome

地中海贫血综合症和异常血红蛋白(简称Hb)是Hb病的两大类。本文主要介绍HbH病和HbE复合β地中海贫血(以下简称HbE-β地贫)两种类型地中海贫血综合征的手术治疗。􀀐

Splenectomy was used to treat two types of thalassemia syndromes in our hospital. Follow-up of thirty-nine cases for 4 months to 5 years was done after splenectomy. Anemia of all patients was improved. Pupils and adults resumed their study and work. Twenty-nine cases with HbH disease and 10 cases with HbE-β thalassemia had an increase in Hb level with an average of 3.5(0.5-7.0)g% and 2.7(0.2-4.3)g% after splenectomy respectively. The differences between those before and after splenectomy were very significant (P<0.001) in both types of diseases. Usually, HbH cases had much better reaction to spleneetomy than HbE-β thalassemia cases. Especially, the patients, who had lower Hb before splenectomy, had higher increase after the operation. They showed a negative correlation (r=-0.864). T Student test showed the dif ferences of Hb level were very significant. Using x and y to be the marker of Hb level before and after spleneetomy respectively, the linear tropic equation is y=8.432-0.874x and the differences of tropic coefficient was obvious (P<0.05). We consider that the equation may be used to evaluate the increase of Hb level in post-splenectomy patients. For example, patients with 8g% Hb may have 1.4g% increase of Hb. But when Hb reach to 9.6g%, it will not increase any more. Thus, we suggest that it is not favorable to perform splenectomy in patients with Hb 8g% or above for incr easing of Hb level.