急性混合表型白血病伴BCR-ABL(P210)阳性的临床分析

Clinical Analysis of Acute Mixed Phenotype Leukemia with Positive BCR-ABL (P210)

目的:探讨急性混合表型白血病(MPAL)伴BCR/ABL融合基因阳性的临床及生物学特性、临床疗效及预后。方法:对重庆市涪陵中心医院确诊的1例患者BCR-ABL (P210)融合基因阳性的急性混合表型白血病的临床特征进行分析,并复习相关文献。结果:通过反转录聚合酶链反应(RT-PCK)和染色体核型分析等方法检测到该例患者存在BCR-ABL P210融合基因及费城染色体(Ph)阳性,给予“达沙替尼”联合静脉药物化疗,疗效差,预后差。结论:Ph+BCR-ABL+混合表型急性白血病患者临床较少见,预后极差,目前尚无标准治疗方案。酪氨酸激酶抑制剂(TKI)联合化疗及造血干细胞移植或CART治疗有可能改善预后。

Objective: To investigate the clinical and biological characteristics, clinical efficacy and prognosis of acute mixed phenotype leukemia(MPAL) with positive BCR/ABL fusion gene. Methods: The clinical features of a patient with BCR-ABL(P210) fusion gene-positive acute mixed phenotype leukemia diagnosed in the hospital were analyzed, and the relevant literature was reviewed. Results: The BCR-ABL P210 fusion gene and Philadelphia chromosome(Ph) positive were detected in this patient by reverse transcription polymerase chain reaction(RT-PCK) and karyotype analysis. “Dasatinib” combined with intravenous drug chemotherapy was given, and its efficacy and prognosis were poor. Conclusion: Patients with Ph+ BCR-ABL+ mixed phenotype acute leukemia are rare in clinical practice and have an extremely poor prognosis. Currently, there is no standard treatment plan. Tyrosine kinase inhibitors(TKI) in combination with chemotherapy and hematopoietic stem cell transplantation or CART have the potential to improve outcomes.