Ph染色体阳性急性巨核细胞白血病1例并文献复习

Philadelphia-chromosome-positive acute megakaryoblastic leukemia:a case report and review of literatures

急性巨核细胞白血病（AMKL）是临床极少见的急性髓系细胞白血病（AML）,国外报道占急性白血病的0.5%-1.2%。其发病呈＂双极＂趋势,儿童发病均在3岁以内,成人的中位发病年龄为58岁,且多见于慢性粒细胞白血病急性变或继发性白血病。Ph染色体阳性原发性AMKL患者报道罕见,我院收治1例原发性Ph染色体阳性AMKL患者并给予甲磺酸伊马替尼治疗,现报告如下。

We reported a case of Philadelphia-chromosome-positive acute megakaryoblastic leukemia（AMKL）and reviewed the literature in order to improve the understanding of cytogenetic,immunologic and clinical characteristics of Philadelphia-chromosome-positive AMKL.Bone marrow morphology,cytogenetic and immunologic examinations of the patient were carried out and the treatment efficacy was observed.The patient was given imatinib and didn＇t achieve hemotological complete remission,finally died in a short time.Philadelphia-chromosomepositive AMKL is a highly heterogeneous disease different from chronic myeloid leukemia,it has some special characteristics in cytogenetic and immunologic,with short survival period and poor prognosis.