仅以血小板增多为首发表现的慢性粒细胞白血病5例临床分析

Clinical study on 5cases of chronic myelogenous leukemia with thrombocythemia onset

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。

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 （〉 1 000 ×10^9/L） , white-cell count and differential count were normal. The megakary0cyte 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.