纯巨核细胞再生障碍性血小板减少症,又称无巨核细胞性血小板减少症(Amegakaryo-cytic Thrombocytopenia),正引起越来越多的血液学工作者的兴趣。它的血液学特征是血小板减少,骨髓巨核细胞减少或缺如,而粒红两系很少异常。本文将对该病的分类、病因、发病机制、实验室资料、诊断及治疗等作一简述。一、分类1985年,Boggs对本病进行了分类。但是,随着造血细胞培养技术的进展,对本病的发病机制的认识亦不断深入。巨核祖细胞内在缺陷、自身免疫等得到证实,合并SLE(系统性红斑狼疮)和类风湿性关节炎也有报道。因此,我们对该分类修改如下: Pure megakaryocyte aplastic thrombocytopenia, also known as Amegakaryo-cytic Thrombocytopenia, is attracting more and more hematological workers. Its hematological characteristics are thrombocytopenia, diminished or absence of bone marrow megakaryocytes, and rare maroon two lines. This article will outline the classification of the disease, etiology, pathogenesis, laboratory data, diagnosis and treatment. First, classification In 1985, Boggs classified the disease. However, with the progress of hematopoietic cell culture technology, the understanding of the pathogenesis of this disease has been deepened. The intrinsic defect of megakaryocyte progenitor cells, autoimmunity, etc. have been confirmed. The combination of SLE (systemic lupus erythematosus) and rheumatoid arthritis has also been reported. Therefore, we modified the classification as follows: