de Montalembert M, Girot R, Revillon Y, Jan D, Adjrad L, Ardjoun F Z, Belhani M, Najean Y
Département de Pédiatrie, Hôpital des Enfants Malades, Paris.
Arch Dis Child. 1990 Mar;65(3):304-7. doi: 10.1136/adc.65.3.304.
Partial splenectomy was performed on 30 patients with homozygous beta thalassaemia to reduce blood requirements and to avoid the risk of overwhelming postsplenectomy infections; 24 patients had thalassaemia major and six thalassaemia intermedia. Five patients received a high transfusion regimen before and after surgery and 25 a lower one. Follow up after surgery ranged from one to four years. Partial splenectomy improved the long term haematological state in the six patients with thalassaemia intermedia. Recurrence of hypersplenism occurred in nine of the 24 patients with thalassaemia major, however, and complete splenectomy was required. Serum IgM concentrations were not significantly modified by surgery. The mean (SD) residual spleen after surgery was 4.45 (2.36) cm measured by scintigraphy. No severe infections occurred after surgery; however, most patients were routinely treated with phenoxymethylpenicillin and the protective effect of the remaining spleen could not be exactly determined. Because of the possibility of recurrence of hypersplenism, routine partial splenectomy when splenectomy is needed in thalassaemia major is not advised, except in children under 5 years whose risk of overwhelming postsplenectomy infection is greatest.
对30例纯合子β地中海贫血患者实施了部分脾切除术，以减少输血需求并避免脾切除术后暴发性感染的风险；其中24例为重型地中海贫血，6例为中间型地中海贫血。5例患者在手术前后接受高输血方案，25例接受低输血方案。术后随访时间为1至4年。部分脾切除术改善了6例中间型地中海贫血患者的长期血液学状态。然而，24例重型地中海贫血患者中有9例出现脾功能亢进复发，需要进行全脾切除术。手术对血清IgM浓度无显著影响。术后通过闪烁扫描测量的残余脾脏平均（标准差）大小为4.45（2.36）cm。术后未发生严重感染；然而，大多数患者常规接受苯氧甲基青霉素治疗，剩余脾脏的保护作用无法准确确定。由于存在脾功能亢进复发的可能性，不建议在重型地中海贫血需要脾切除时常规进行部分脾切除术，5岁以下儿童除外，因为他们发生脾切除术后暴发性感染的风险最大。
