Ph阴性青少年与年轻成人急性淋巴细胞白血病患者的治疗存在较多争议。标危组患者可以通过儿童样化疗方案获益。儿童方案优于成人方案的原因包括了这类患者的遗传学特点,治疗中应用非骨髓抑制类药物,更高剂量的氨甲蝶呤以及更高强度的中枢神经系统白血病预防与治疗。高危组患者则可通过异基因造血干细胞移植获益。研究显示MEF2D融合基因、Ph样急性淋巴细胞白血病为预后不良因素。本文就Ph阴性青少年与年轻成人急性淋巴细胞白血病的预后及治疗研究进展进行综述。
There is a general debate about the treatment strategy of Ph negative adolescent and young adult acute lymphoblastic leukemia. Clinical researches show the patients in the standard risk group can benefit from the pediatric-inspired protocol. Several reasons may contribute to the superiority of the pediatric inspired protocol to the adult protocol,including the genetic characteristics of these patients,using non-myelosuppression agents,higher dose of Methotrexate,and more intensive CNS for prophylaxis and treatment. Patients in the high risk group can benefit from the Allo-HSCT. Furthermore,MEF2 D fusion gene and Ph-like ALL are shown to be the risk factors for poor prognosis. In this article,we will review the research advances of the prognosis and treatment strategy of this disease.