免疫相关性血/全血细胞减少症诊断和治疗  

Diagnosis and treatment of immuno-related hemocytopenia/pancytopenia

作　　者：王一浩[1] 付蓉[1] 邵宗鸿[1] WANG Yi-hao;FU Rong;SHAO Zong-hong(Department of Hematology,Tianjin Medical Universiyt General Hospital,Tianjin 300052,China)

机构地区：[1]天津医科大学总医院血液科,天津300052

出　　处：《中国实用内科杂志》2022年第7期540-545,共6页Chinese Journal of Practical Internal Medicine

基　　金：国家自然科学基金(81900131,81970116);天津市自然科学科学基金(18JCQNJC80400);天津市科委研究基金(2018KJ043);天津市科学技术规划基金(20YFZCSY00060);天津医科大学基金(2010ky20);天津市卫生局科技基金(2010KZ105)。

摘　　要：免疫相关性血/全血细胞减少症(IRH/IRP)是一种自身抗体介导的骨髓造血功能衰竭性疾病,常常被误诊为“不典型或难治性再生障碍性贫血”或“骨髓增生异常综合症-难治性贫血”,造血细胞自身抗原耐受丧失到自身抗体产生并攻击自体骨髓造血的发病过程,是免疫细胞“瀑布链”异常激活所致。识别其异常体液免疫功能亢进的发病本质是IRH/IRP精准诊治的关键。Immune-related hemocytopenia/pancytopenia(IRH/IRP)is one kind of bone marrow hematopoietic failure disease caused by autoantibodies.It is often misdiagnosed as"refractory aplastic anemia"or"myelodysplastic syndrome-refractory anemia".Independent pathogenesis,in which autoantibodies resulted from loss of autoantigen tolerance of hematopoietic cell was produced and attacked targeted antigens,was involved in abnormal activation of a series of immune cells.Precise diagnosis and targeted therapy of IRH/IRP based on identification abnormal hyperfunction of humoral immunity and increased autoantibodies.

关 键 词：免疫相关性血细胞减少 诊断 治疗 

分 类 号：R552[医药卫生—血液循环系统疾病]