Clinical study on 5cases of chronic myelogenous leukemia with thrombocythemia onset
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摘要: 目的:探讨仅以血小板增多为首发而没有典型慢性粒细胞白血病(CML)表现的5例患者的临床诊断及治疗效果。方法:回顾性分析5例仅以血小板增多为首发表现的CML患者的临床资料。结果:5例患者均仅以血小板增多为首发表现,均无肝脾肿大;血小板均>1 000×109/L,白细胞计数及分类正常;骨髓细胞学示巨核细胞明显增多,粒系增生正常,NAP积分正常或增加;患者均有Ph染色体和BCR-ABL融合基因;患者均使用伊马替尼治疗,4例在12个月内达到完全分子学反应。结论:仅以血小板增多为首发表现的患者,如缺乏典型CML的临床表现,易误诊为原发性血小板增多症,应作Ph染色体及BCR-ABL融合基因检测以鉴别;这些患者给予伊马替尼治疗取得很好的疗效,可能是CML的发病早期。
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关键词:
- 血小板增多 /
- 慢性粒细胞白血病 /
- Ph染色体 /
- BCR-ABL融合基因
Abstract: Objective:To evaluate the diagnosis and therapeutic effect of chronic myelogenous leukemia (CML) with thrombocythemia onset.Method:The clinical manifestations,laboratory features,and treatment in 5 cases of CML with thrombocythemia onset were analyzed retrospectively.Result:All patients were only thrombocythemia onset,and splenomegaly was not present in all cases.Platelet significantly increased (>1000×109/L),white-cell count and differential count were normal.The megakaryocyte increased obviously,granulopoiesis and maturation were normal,neutrophil alkaline phosphatase was normal or slight increased in bone marrow.Ph chromosome and BCR-ABL fusion gene were present in all cases.All patients were treated with imatinib,4 cases achieved complete molecular response within 12 months.Conclusion:CML with thrombocythemia onset,while lack of classic clinical features of CML,is easily misdiagnosed as essential thrombocythemia and should be detected Ph chromosome and BCR-ABL fusion gene.These observations indicated CML with thrombocythemia onset can be regarded as early manifestations of the chronic stable phase. -
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