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[脾切除术治疗常见可变免疫缺陷合并血细胞减少症:1例报告及文献复习]

[Splenectomy for the treatment of common variable immunodeficiency complicated with cytopenia: report of one case and literature review].

作者信息

Qu C Y, Liu W, Fu R F, Chen Y F, Liu X F, Zhang L, Yang R C, Xue F

机构信息

State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Tianjin 300020, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2021 Oct 14;42(10):846-850. doi: 10.3760/cma.j.issn.0253-2727.2021.10.009.

Abstract

To improve the understanding of splenectomy for treating common variable immunodeficiency complicated with cytopenia. A case of common variable immunodeficiency complicated with cytopenia was reported, and the literature was reviewed. The patient, female, 16 years old, was hospitalized for eight years due to thrombocytopenia; she manifested recurrent thrombocytopenia with leukopenia since adolescence. The patient was diagnosed with common variable immunodeficiency with repeated mild infections, splenomegaly, and significantly reduced plasma immunoglobulin levels. Additionally, splenectomy was performed with adequate immunoglobulin replacement therapy, and the pathology confirmed hypersplenism; her blood cell level returned to normal after surgery. Common variable immunodeficiency has various clinical manifestations and can be complicated with cytopenia. Under the premise of adequate immunoglobulin replacement therapy, splenectomy is a safe and effective treatment for common variable immunodeficiency in patients with recurrent cytopenia.

摘要

为提高对脾切除术治疗常见变异型免疫缺陷合并血细胞减少症的认识。报告1例常见变异型免疫缺陷合并血细胞减少症的病例,并复习相关文献。患者为16岁女性,因血小板减少症住院8年;自青春期起反复出现血小板减少伴白细胞减少。该患者被诊断为常见变异型免疫缺陷,伴有反复轻度感染、脾肿大和血浆免疫球蛋白水平显著降低。此外,在进行充分的免疫球蛋白替代治疗的情况下实施了脾切除术,病理证实为脾功能亢进;术后血细胞水平恢复正常。常见变异型免疫缺陷有多种临床表现,可合并血细胞减少症。在充分的免疫球蛋白替代治疗的前提下,脾切除术是治疗反复出现血细胞减少症的常见变异型免疫缺陷患者的一种安全有效的治疗方法。

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