再生障碍性贫血的排除诊断

Exclusive diagnosis of aplastic anemia

作为一种独立疾病,再生障碍性贫血(AA)已被人们认识一个多世纪了,至今确诊该病仍需排除其他骨髓衰竭性疾病,即排除诊断。因为目前还没有一个参数可以敏感、特异地诊断AA,排除诊断实属无奈。因此,应进一步寻找AA有意义的诊断参数,以建立该疾病的直接诊断方案,并使其与其他骨髓衰竭疾病相鉴别,如先天性骨髓衰竭、低增生骨髓增生异常综合征、AA-阵发性睡眠性血红蛋白尿综合征、大颗粒淋巴细胞白血病、意义不明的克隆性血细胞减少症、免疫相关性全血细胞减少症、急性造血阻滞和意义不明的特发性血细胞减少症。有助于鉴别AA与其他骨髓衰竭的新型标志物和技术应该用于AA的诊断。对排除诊断认识与应用正确与否,不仅关系到被排除疾病诊治正确性,更关系到AA诊断、治疗和研究质量。

As an independent disease,aplastic anemia(AA)has been recognized for more than a century.When AA is diagnosed,other non-AA bone marrow failures should be excluded.It is termed as exclusive diagnosis of AA.The exclusive diagnosis of AA is helplessly based on that there is no parameter by which AA can be sensitively and specifically diagnosed now.So further searching for the meaningful diagnostic parameters of AA should be carried on to establish a direct diagnostic protocol of this disease and make it possible to differentiate it clearly from other bone marrow failure disease such as congenenital bone marrow failure,hypoplastic myelodysplastic syndromes,AA-paroxysmal nocturnal hemoglobinuria syndromes,large granules lymphocyte leukemia,clonal cytopenia of undetermined significance,immunorelated pancytopenia,acute hemopoietic arresting and idiopathic cytopenia of undetermined significance.The new markers and technologies being helpful for distinguishing AA from other bone marrow failures should be used in diagnosing AA.Correct understanding and application of exclusive diagnosis is not only related to the correctness of diagnosis and treatment of excluded diseases,but also to the quality of AA diagnosis,treatment and research.