脾切除治疗普通变异型免疫缺陷病合并血细胞减少一例报告并文献复习  

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

作　　者：曲翠云 刘葳 付荣凤 陈云飞 刘晓帆 张磊 杨仁池 薛峰 Qu Cuiyun;Liu Wei;Fu Rongfeng;Chen Yunfei;Liu Xiaofan;Zhang Lei;Yang Renchi;Xue Feng(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)

机构地区：[1]中国医学科学院血液病医院(中国医学科学院血液学研究所),实验血液学国家重点实验室,国家血液系统疾病临床医学研究中心,天津300020

出　　处：《中华血液学杂志》2021年第10期846-850,共5页Chinese Journal of Hematology

基　　金：国家自然科学基金(82070125)。

摘　　要：目的提高对脾切除术治疗普通变异型免疫缺陷病合并血细胞减少的认识。方法报告1例脾切除治疗普通变异型免疫缺陷病合并血细胞减少病例并进行文献复习。结果患者,女,16岁,因发现血小板减少8年入院。患者于青少年期发病,以反复血小板减少伴白细胞减少为主,感染症状较轻,脾肿大,血浆免疫球蛋白水平明显减低,诊断为普通变异型免疫缺陷病。给予足量静脉注射免疫球蛋白替代治疗后行脾切除术,脾脏病理示脾功能亢进,术后血细胞恢复正常水平。结论普通变异型免疫缺陷病临床表现多样,可合并脾肿大、血细胞减少,在足量静脉注射免疫球蛋白替代治疗的前提下,对于反复血细胞减少的脾肿大患者,脾切除是一种安全有效的治疗方式。Objective To improve the understanding of splenectomy for treating common variable immunodeficiency complicated with cytopenia.Methods A case of common variable immunodeficiency complicated with cytopenia was reported,and the literature was reviewed.Results 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.Conclusions 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.

关 键 词：普通变异型免疫缺陷病 血细胞减少 脾切除 

分 类 号：R657.6[医药卫生—外科学]