Raza A, Minowada J, Barcos M, Rakowski I, Preisler H D
Eur J Cancer Clin Oncol. 1984 Dec;20(12):1509-16. doi: 10.1016/0277-5379(84)90144-5.
Twenty-five cases of Ph1-positive acute leukemia (AL) are described, 13 presenting an acute lymphocytic leukemia (ALL) and 12 an acute non-lymphocytic leukemia (ANLL). In 12 cases coarse, pink, peroxidase-negative cytoplasmic granules were found in the leukemic cells. These granules have not been described in Ph1-negative AL and their presence appears to be pathognomonic of Ph1-positive acute leukemia. The leukemias of three patients consisted of both lymphoid and myeloid clones while the cells of two patients had lymphoid and myeloid markers simultaneously present in the same cells. Cytogenetic studies were useful for monitoring response and some patients clearly acquired a Ph1-negative status during clinical remission. The disease appears to be more resistant to chemotherapy than Ph1-negative acute leukemia. While similar to chronic myelocytic leukemia (CML) in the Ph1 translocation, Ph1 AL differed from it both in age at presentation and response to therapy.
本文描述了25例Ph1阳性急性白血病(AL)患者,其中13例为急性淋巴细胞白血病(ALL),12例为急性非淋巴细胞白血病(ANLL)。在12例患者的白血病细胞中发现了粗大、粉红色、过氧化物酶阴性的细胞质颗粒。这些颗粒在Ph1阴性AL中未见报道,它们的存在似乎是Ph1阳性急性白血病的特征性表现。3例患者的白血病由淋巴样和髓样克隆组成,而2例患者的细胞在同一细胞中同时存在淋巴样和髓样标志物。细胞遗传学研究有助于监测反应,一些患者在临床缓解期明显获得了Ph1阴性状态。该疾病似乎比Ph1阴性急性白血病对化疗更具抗性。虽然Ph1阳性急性白血病在Ph1易位方面与慢性粒细胞白血病(CML)相似,但在发病年龄和对治疗的反应方面与CML不同。