Introduction: Splenomegaly and hypersplenism are common complications of thalassemia patients due to the excessive clearance of defective red blood cells from the spleen. To date, splenectomy has been considered one of the most effective treatments for splenomegaly, reducing clinical severity among thalassemia patients. Thus, we aim to investigate the differences in splenectomy rates and hematological indices among thalassemia patients with different genotypes. Method: In this study, we analyzed the clinical data of thalassemia in 2,130 patients admitted to the 923rd Hospital of the People's Liberation Army from January 2006 to December 2020, and the statistical software SPSS 26.0 was applied to analyze the data. Result: Of the 2,130 patients with thalassemia, 265 patients underwent splenectomy. It was determined that significantly more patients with hemoglobin H (HbH) disease, a form of alpha-thalassemia, have undergone splenectomy than beta-thalassemia patients (20% vs. 7%). Further, HbH disease patients were diagnosed at a significantly older age than beta-thalassemia patients. Conclusion: The greater probability of HbH disease patients undergoing splenectomy is likely influenced by multiple factors, including their lower dependency on transfusion, leading to high spleen compensatory stress on the spleen, and the destruction of defective erythrocytes. In contrast, beta-thalassemia is clinically more severe and less tolerant of hemoglobin fluctuations. Based on these findings, clinicians are suggested to pay more attention to HbH disease patients as many of them are still under-transfused, which could lead to chronic hemolysis and more severe hepatosplenomegaly. These results might offer insight for improving the clinical management of patients with different types of thalassemia. (c) 2023 S. Karger AG, Basel

我国地中海贫血(简称地贫)患者中数量最多的是中间型α地贫,也称血红蛋白H病(hemoglo-bin H disease,Hb H病)[1],由于未得到足够重视,该类型患者的生存状况甚至比不上规范高量输血的重型β地贫(thalassaemia major,TM)[2-3].近年来,国际上根据临床严重程度和输血需求将地贫分为输血依赖型地贫(transfusion dependent thalassaemia,TDT)和非输血依赖型地贫(non transfusion dependent thalassaemia,NTDT)两类,并制定了相应的管理指南[2-4].部分Hb H病患者属于TDT,需规范高量输血和铁螯合剂治疗,这些患者中部分是发病时即为TDT,部分是后来出现并发症或贫血加重转为TDT的[2-3,5-6].本文基于笔者所在医疗单位收治的病例,略述Hb H病的治疗.

血红蛋白H病(hemoglobin H disease,Hb H病)患者是我国地中海贫血(地贫)患者中人数最多的群体.Hb H病临床表现差异极大,是地贫高发区遗传咨询工作中最难处理的类型,也是临床医师制定治疗方案时难以应对的类型.