Wolf D J, Silver R T, Coleman M
Cancer. 1978 Oct;42(4):1957-63. doi: 10.1002/1097-0142(197810)42:4<1957::aid-cncr2820420439>3.0.co;2-2.
Four patients who demonstrated unusually prolonged survival with Philadelphia chromosome positive Ph' (+) chronic myeloid leukemia (CML) were analyzed for factors associated with survival. Survival duration from initial diagnosis ranged from 120 to 222 months, with a mean of 170 months. At diagnosis, age, symptoms, liver or spleen size, hematocrit, white blood cell count, absolute peripheral myeloblast plus promyelocyte count, and uric acid did not have unique prognostic significance. At diagnosis all four patients had normal or low-normal platelet counts, (range: 170,000 to 248,000/mm3). Thrombocytopenia occurred during treatment in three patients. None of the four patients, however, developed severe marrow hypoplasia or leukopenia during treatment for the chronic phase. Cytogenic studies performed from 103 to 156 months after diagnosis did not reveal a large subpopulation of marrow cells with a normal karyotype or cells with the XO genotype in the male patients. These observations suggest that prolonged survival in CML 1) is not contingent upon intensive treatment resulting in marrow hypoplasia, and 2) does not require the persistence of a clone of karyotypically-normal bone marrow cells or a clone of marrow cells in males which has lost the Y chromosome. A normal or low-normal platelet count at diagnosis may be a favorable prognostic indicator.
对4例费城染色体阳性（Ph'（+））慢性髓性白血病（CML）患者生存期异常延长的情况进行了分析，以寻找与生存相关的因素。从初次诊断开始的生存期为120至222个月，平均为170个月。诊断时，年龄、症状、肝脾大小、血细胞比容、白细胞计数、外周血原始粒细胞加早幼粒细胞绝对计数以及尿酸均无独特的预后意义。诊断时，所有4例患者的血小板计数均正常或略低于正常（范围：170,000至248,000/mm³）。3例患者在治疗期间出现血小板减少。然而，4例患者在慢性期治疗期间均未出现严重的骨髓发育不全或白细胞减少。诊断后103至156个月进行的细胞遗传学研究未发现男性患者中存在大量核型正常的骨髓细胞亚群或具有XO基因型的细胞。这些观察结果表明，CML患者生存期延长1）并非取决于导致骨髓发育不全的强化治疗，2）也不需要核型正常的骨髓细胞克隆或男性中丢失Y染色体的骨髓细胞克隆持续存在。诊断时血小板计数正常或略低于正常可能是一个有利的预后指标。
