Department of Pediatrics, 923rd Hospital of the People's Liberation Army, Nanning, China.
Office of Clinical Trial Institute, Jiangbin Hospital of Guangxi Zhuang Autonomous Region, Nanning, China.
Acta Haematol. 2023;146(6):458-464. doi: 10.1159/000533233. Epub 2023 Aug 11.
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.
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.
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 α-thalassemia, have undergone splenectomy than β-thalassemia patients (20% vs. 7%). Further, HbH disease patients were diagnosed at a significantly older age than β-thalassemia patients.
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, β-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.
由于脾脏过度清除缺陷红细胞，地中海贫血患者常出现脾肿大和脾功能亢进。迄今为止，脾切除术已被认为是治疗脾肿大最有效的方法之一，可以降低地中海贫血患者的临床严重程度。因此，我们旨在研究不同基因型地中海贫血患者脾切除率和血液学指标的差异。
本研究分析了 2006 年 1 月至 2020 年 12 月期间 2130 例解放军 923 医院收治的地中海贫血患者的临床资料，应用统计软件 SPSS 26.0 进行数据分析。
在 2130 例地中海贫血患者中，265 例行脾切除术。结果表明，血红蛋白 H（HbH）病患者（一种α地中海贫血）行脾切除术的比例明显高于β地中海贫血患者（20%比 7%）。此外，HbH 病患者的诊断年龄明显大于β地中海贫血患者。
HbH 病患者行脾切除术的可能性更大，可能受到多种因素的影响，包括对输血的依赖性较低，导致脾脏代偿性压力增加，以及缺陷红细胞的破坏。相比之下，β地中海贫血的临床症状更为严重，对血红蛋白波动的耐受性较低。基于这些发现，建议临床医生更加关注 HbH 病患者，因为许多患者仍未接受足够的输血治疗，这可能导致慢性溶血和更严重的肝脾肿大。这些结果可能为改善不同类型地中海贫血患者的临床管理提供参考。
