骨髓衰竭症的精准鉴别  

Accurate identification of bone marrow failure syndromes

作　　者：付蓉[1] FU Rong(Department of Hematology,Tianjin Medical University General Hospital,Tianjin 300052,China)

机构地区：[1]天津医科大学总医院血液内科,天津300052

出　　处：《中国实用内科杂志》2022年第10期824-830,共7页Chinese Journal of Practical Internal Medicine

基　　金：国家自然科学基金(81970115);天津市科技计划(21JCZDJC01180);天津市医学重点学科(专科)建设项目资助。

摘　　要：骨髓衰竭症(BMFS)是一组骨髓造血功能低下导致的全血细胞减少症候群。该类疾病取材困难,病理机制复杂,诊断和鉴别诊断缺乏特异指标,临床表现雷同,治疗手段相对不足。目前诊断再生障碍性贫血(AA)需要除外骨髓增生异常综合征(MDS),阵发性睡眠性血红蛋白尿症(PNH)等,而诊断MDS也需除外AA。这种“相互排除诊断”,造成了不同骨髓衰竭性疾病之间的复杂关系,造成了逻辑上的混乱,而只有明晰骨髓衰竭类型,才能实施更精准的治疗,以提升疗效。文章将从骨髓衰竭性疾病的认识历程、分类、每种独立疾病的特点及鉴别要点做一评述。Bone marrow failure syndromes(BMFS)is a group of syndromes with pancytopenia caused by low hematopoietic function of bone marrow.It is difficult to obtain materials and the pathological mechanism of these diseases is complex,and the diagnosis and differential diagnosis lack specific indicators. In addition,the clinical manifestations of these diseases are similar,and the treatment methods are relatively insufficient.At present,the diagnosis of aplastic anemia(AA)needs to exclude myelodysplastic syndrome(MDS),paroxysmal nocturnal hemoglobinuria(PNH),and the diagnosis of MDS also needs to exclude AA.This "mutual exclusion diagnosis" has caused a complex relationship between different BMFs and caused logical confusion.We can implement more precise treatment to improve the efficacy only by clarifying the type of BMFs.Therefore,this article will reviewthe understanding process,classification,characteristics and identification points of BMFs.

关 键 词：骨髓衰竭症 鉴别 精准诊断 

分 类 号：R551.3[医药卫生—血液循环系统疾病]