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25年前引入白消安治疗以来,慢性粒细胞白血病(CGL)的预后迄今无明显改善。其原因部分由于力量集中在更为引人注目的急性白血病上,并且对CGL有不在专科中心进行治疗之倾向。不过,主要的原因则在于CGL生物学。该病易于控制的慢性期终结转化到难治期,治疗很少能推迟这种结局或于发生后延长寿命,CGL的中数生存期变动不大。(命名法)对CGL的难治期命名是不完善的。正象很多对疾病较旧的描述,“原始细胞危象”一词,仅是从现今所知的某些病例特征中挑选出的明显的特征,不能包罗万象。“急性转化”也不恰当,因为存在的很多变型并非急性的。Baikie采用“恶变”(metamorphosis)来包括所有CGL慢性期的转化,其精确的变型可借细胞学形态进一步确定,正如在非何杰金氏淋巴瘤中那样。(流行病学)发病率;CGL恶变的类型复杂莫测,其确切发病率尚不肯定。有些患者死于治疗的作用,大约有15%(依据所观察的病例的年
The prognosis of chronic myeloid leukemia (CGL) has not improved significantly so far since the introduction of busulfan 25 years ago. The reason is partly due to the concentration of power on the more striking acute leukemias and the tendency of CGLs not to be treated in specialized centers. However, the main reason is the CGL biology. The disease is easily controlled in the chronic phase of end-to-end conversion to refractory disease, treatment rarely delays this outcome or prolongs life after it occurs, and changes in the median survival of CGL little. (Nomenclature) The refractory term to CGL is not perfect. Like many older descriptions of the disease, the term “primitive cell crisis” is only a salient feature that can be picked out of some of the case characteristics known to date and can not be comprehensive. “Acute transformation” is not appropriate because many of the variants that exist are not acute. Baikie used “metamorphosis” to include all CGL chronic phase transformations, and exact variants of which can be further defined by cytology, as in non-Hodgkin’s lymphoma. (Epidemiology) incidence; the type of CGL malignant complex unpredictable, the exact incidence is uncertain. Some patients die from the effects of treatment, about 15% (based on the observed years of the case