bcr-abl融合基因阳性原发性血小板增多症一例报道并文献复习

bcr-abl gene positive essential thrombocythemia: one case report and literature review

目的提高对bcr-abl融合基因阳性原发性血小板增多症(essentialthrombocythemia,ET)的认识。方法报告1例bcr-abl融合基因阳性ET(bcr-abl+ET)并进行文献复习。结果bcr-abl+ET具有女性多见、脾脏不大或仅轻度增大、外周血白细胞大多<40×109/L、嗜碱粒细胞常不增多,幼稚粒细胞少见、骨髓有核细胞增生以粒系和巨核系两系或单系巨核系增生为主、中性粒细胞碱性磷酸酶积分正常或增高等特点。结论bcr-abl+ET有别于经典的慢性粒细胞白血病及ET,可能是ET的一种新的变异型。

Objective To improve the recognition of bcr-abl gene positive essential thrombocythemia (ET). Methods A case of bcr-abl gene positive ET was reported and the related literature was reviewed. Results The bcr-abl+ ET had following characters: female predominance, milder or no splenomegaly, peripheral leukocytes count <40 ×109/L , less or no basophilia and fewer immature granulocytes in peripheral blood, bone marrow granulocytic and/ or megakaryocytic lineage hyperplasia and normal or increased neutrophil a...