bcr-abl基因阳性血小板增多症八例报告——附文献复习  

Report of 8 cases of bcr-abl gene positive thrombocytosis and review of the li terature

作　　者：林凤茹[1] 王艳[1] 陈静[1] 

机构地区：[1]河北医科大学第二医院血液内科,石家庄050000

出　　处：《中华血液学杂志》2004年第9期528-531,共4页Chinese Journal of Hematology

摘　　要：目的　分析 8例bcr abl融合基因阳性 (Bcr+ )血小板增多症特点并对其归属提出探讨意见。方法　回顾性分析 8例Bcr+ 血小板增多症患者的临床、血液学、治疗转归 ,并与经典原发性血小板增多症 (ET)和血小板增多的慢性粒细胞白血病慢性期 (CML CP T)比较其异同 ,以PCR法检查bcr abl融合基因。结果　除bcr abl融合基因外 ,Bcr+ 血小板增多症在临床、血液学、治疗转归与ET无明显差异 ;与CML CP T不同在于 :女性较多 ,脾脏多不肿大或轻度肿大 ,外周血白细胞增高 ,但 <4 0× 10 9/L ,嗜碱粒细胞不增高 ,幼稚粒细胞少见 ,骨髓有核细胞以粒、巨核两系或单巨核系增生 ,中性粒细胞碱性磷酸酶 (NAP)积分正常或增高 ,少有急性转化。结论　Bcr+ 血小板增多症作为慢性骨髓增殖性疾病新的一员变异型ET ,尚需进一步研究。Objective To analyse the features of 8 cases of Bcr+ thrombocytosis. Methods The clinical and hematological features and therapeutic outcomes were studied retrospectively in 8 Bcr+ thrombocytosis and compared with essential thromboc ytosis (ET) and chronic myeloid leukemia-chronic phase thrombocytosis (CML-CP -T). BCR-ABL fusion gene was detected with PCR. Results ① Exc ept for the presence of BCR-ABL fusion gene, there was no significant differenc e in clinical and hematological features and therapeutic outcomes between thromb ocytosis with or without BCR-ABL. ② The Bcr+ thrombocytosis differed from CM L-CP-T in the following aspects: female predominance, milder or no splenomegal y, peripheral leukocytes count <40×109/L, less or no basophilia and fewer i mmature granulocytes in peripheral blood, bone marrow granulocytic and/or megaka ryocytic lineage hyperplasia, normal or increased neutrophil alkaline phosphatas e score and less blastic transformation. Conclusion Bcr+ thrombo cytosis may be considered as a new member of chronic myeloproliferative diseases , a variant of essential thrombocythemia.

关 键 词：血小板增多症 BCR-ABL融合基因 治疗 ET 阳性 增高 BCR-ABL基因 性转化 PCR法 血液学 

分 类 号：R558.3[医药卫生—血液循环系统疾病]