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成人中间型β地中海贫血的部分脾切除术治疗:一例报告

Partial Splenectomy in the treatment of an adult with β thalassemia intermedia: A case report.

作者信息

Correia João Guardado, Moreira Nídia, Costa Almeida Carlos Eduardo, Reis Luís Simões

机构信息

Instituto Português de Oncologia de Coimbra Francisco Gentil, Av. Bissaya Barreto 98, 3000-075 Coimbra, Portugal.

Centro Hospitalar e Universitário de Coimbra (Hospital Geral - Covões), S. Martinho de Bispo, 3041-853 Coimbra, Portugal.

出版信息

Int J Surg Case Rep. 2017 Nov 11;41:446-449. doi: 10.1016/j.ijscr.2017.11.011. eCollection 2017.

Abstract

INTRODUCTION

Thalassemia is a common disease which treatment is often based on splenectomy. The risks associated with total splenectomy stimulated partial splenectomy as a potentially alternative therapy.

CASE PRESENTATION

A 45 year-old female patient with long term follow-up for β thalassemia intermedia started to develop signs of hypersplenism and iron overload. A partial splenectomy was performed and was observed a marked hematologic improvement while preserving the desired splenic function.

DISCUSSION

Partial splenectomy proved to provide a persistent decrease in hemolytic rate while preserving the integrity of splenic phagocytic function, presenting itself as an effective alternative to total splenectomy. After being subjected to partial splenectomy, our patient experienced a sustained control of hemolysis and showed no signs of hypersplenism or iron overload. No splenic regrowth or infectious complications were observed. The major drawbacks of partial splenectomy are the increased risk of intra- and postoperative bleeding, splenic remnant torsion and splenic regrowth.

CONCLUSION

Partial splenectomy is an alternative to total splenectomy for the treatment of adult β Thalassemia intermedia patients avoiding the risks associated with total splenectomy.

摘要

引言

地中海贫血是一种常见疾病,其治疗通常基于脾切除术。与全脾切除术相关的风险促使部分脾切除术成为一种潜在的替代疗法。

病例介绍

一名45岁的中间型β地中海贫血女性患者,长期随访,开始出现脾功能亢进和铁过载迹象。进行了部分脾切除术,观察到血液学有显著改善,同时保留了所需的脾功能。

讨论

部分脾切除术被证明可使溶血率持续降低,同时保留脾吞噬功能的完整性,是全脾切除术的有效替代方法。接受部分脾切除术后,我们的患者溶血得到持续控制,未出现脾功能亢进或铁过载迹象。未观察到脾再生或感染并发症。部分脾切除术的主要缺点是术中和术后出血风险增加、脾残余扭转和脾再生。

结论

部分脾切除术是治疗成年中间型β地中海贫血患者全脾切除术的替代方法,可避免与全脾切除术相关的风险。

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