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对血液学家来说,有效地治疗慢性粒细胞白血病(CGL)仍是一个棘手的问题。虽然对治疗Ph~1染色体阳性的CGL患者能使其生命状况有所改善,但是用目前的治疗方案对患者的生存期改进不大。CGL在儿童白血病中只占2%～4%,而Ph~1阳性者更为罕见。在成人CGL患者中,Ph~1阴性者占10%～20%,这些患者发病的中数年龄为60岁,他们的生存期达18个月。如果儿童发生这种类型的白血病,则对化疗反应更差,且在几个月内就死亡。这种所谓的幼年型CGL与Ph~1阳性的GGL在临床方面有本质上的不同。在试管中的骨髓培养结果是纯粹的单核细胞集落,而不象Ph~1阳性的CGL患者是粒细胞集落,这就提示它可能是急性单核细胞性白血病的一种类型。患此病的儿童多半在4岁
For hematologists, the effective treatment of chronic myelogenous leukemia (CGL) remains a thorny issue. Although CGL patients who treat Ph-1-positive CGLs have improved their life status, the current treatment regimen does not improve patient survival significantly. CGL accounts for only 2% to 4% of childhood leukemia, whereas Ph-1 positive is even more rare. Among adult CGL patients, Ph-1 negatives account for 10% to 20%. The median age at onset of these patients is 60 years and their survival is 18 months. If this type of leukemia occurs in children, the response to chemotherapy is worse, and death occurs within a few months. This so-called juvenile CGL has a clinically significant difference from Ph-1-positive GGL. Bone marrow cultures in test tubes resulted in pure monocyte colonies, whereas those with Ph ~ 1-positive CGL were granulocyte colonies, suggesting that it may be a type of acute monocytic leukemia. Children with the disease are mostly 4 years old