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[中间型β地中海贫血合并严重溶血性贫血患者的脾切除术]

[Splenectomy in a patient with beta thalassemia intermedia and severe hemolytic anemia].

作者信息

Pérez J A, Padilla J, Rodríguez M A, Cura S, Sánchez S, Cutillas M J, Sanz J

机构信息

Servicio de Anestesiología y Reanimación, Hospital General Universitario José M. Morales Meseguer, Murcia, Spain.

出版信息

Rev Esp Anestesiol Reanim. 2001 Jun-Jul;48(6):288-91.

PMID:11446945
Abstract

A 36-year-old woman with beta thalassemia intermedia suffered a hemolytic crisis secondary to Epstein-Barr virus infection. She was given a transfusion of phenotypically compatible blood. However, severe hemolysis persisted (with hemoglobin levels around 4 g/dl) in spite of gamma globulin and corticosteroid administration. Emergency therapeutic splenectomy was performed. We discuss intraoperative management in situations of severe anemia, concluding that it is possible to survive significant decreases in hemoglobin concentration with cardiocirculatory reserve and respiration intact, maintaining normal volume.

摘要

一名36岁的中间型β地中海贫血女性患者因感染爱泼斯坦-巴尔病毒而引发溶血性危机。她接受了表型匹配的输血。然而,尽管给予了γ球蛋白和皮质类固醇治疗,严重溶血仍持续存在(血红蛋白水平约为4 g/dl)。遂进行了急诊治疗性脾切除术。我们讨论了严重贫血情况下的术中管理,得出结论:在心脏循环储备和呼吸功能完好、维持正常血容量的情况下,血红蛋白浓度显著下降时仍有可能存活。

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引用本文的文献

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Hemolytic Anemia Linked to Epstein-Barr Virus Infectious Mononucleosis: A Systematic Review of the Literature.与爱泼斯坦-巴尔病毒感染性单核细胞增多症相关的溶血性贫血:文献系统综述
J Clin Med. 2025 Feb 15;14(4):1283. doi: 10.3390/jcm14041283.
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Anaesthetic management of beta thalassemia major with hypersplenism for splenectomy in pediatric age group: Report of four cases.小儿重型β地中海贫血伴脾功能亢进行脾切除术的麻醉管理:4例报告
Anesth Essays Res. 2015 May-Aug;9(2):266-9. doi: 10.4103/0259-1162.156362.