Cortes J E, Talpaz M, Beran M, O'Brien S M, Rios M B, Stass S, Kantarjian H M
Department of Hematology, University of Texas M.D. Anderson Cancer Center, Houston 77030.
Cancer. 1995 Jan 15;75(2):464-70. doi: 10.1002/1097-0142(19950115)75:2<464::aid-cncr2820750209>3.0.co;2-e.
Five to 10% of patients with chronic myelogenous leukemia (CML) do not have the Philadelphia chromosome (Ph), but one-third of them have rearrangements of the breakpoint cluster region (BCR-positive).
The authors analyzed the characteristics, treatment response, and prognosis of 23 patients with BCR-positive, Ph-negative CML, and compared them with patients with Ph-positive CML, Ph-negative BCR-negative CML and chronic myelomonocytic leukemia (CMML) treated during the same period.
Seventeen patients had early chronic phase CML, 3 had late chronic phase, 2 had accelerated phase, and 1 had blastic phase. The median age was 44 years (range, 14-71 years), median platelet count was 402 x 10(9)/l, and median leukocyte count was 86 x 10(9)/l. Fourteen of the 17 patients with early chronic phase CML received alpha-interferon; 12 (86%) achieved complete hematologic remission. Median survival in chronic phase CML was 60 months (range, 3-90+ months). Patients with Ph-negative BCR-positive CML and those with Ph-positive CML had similar characteristics and outcome. Compared with patients with Ph-negative BCR-negative CML and CMML, patients with Ph-negative BCR-positive CML and Ph-positive CML were significantly younger, had a significantly higher incidence of leukocytosis, thrombocytosis, and peripheral and marrow basophilia, and a significantly lower incidence of anemia, thrombocytopenia, marrow blast percent, and peripheral and marrow monocytosis. The median survival was 60 months for Ph-negative BCR-positive CML, 73 months for Ph-positive CML, 25 months for Ph-negative BCR-negative CML, and 9 months for CMML (P < 0.001). When analyzed adjusting for their stage, patients classified with Ph-negative BCR-positive CML. Stage I disease had a significantly better survival than did patients with Ph-negative BCR-negative CML (P < 0.02).
Patients with Ph-negative BCR-positive CML are similar to those with Ph-positive CML and should be treated with the same approaches.
5%至10%的慢性粒细胞白血病（CML）患者没有费城染色体（Ph），但其中三分之一有断裂簇区域重排（BCR阳性）。
作者分析了23例BCR阳性、Ph阴性CML患者的特征、治疗反应和预后，并将他们与同期治疗的Ph阳性CML、Ph阴性BCR阴性CML和慢性粒单核细胞白血病（CMML）患者进行比较。
17例患者处于慢性期早期CML，3例处于慢性期晚期，2例处于加速期，1例处于急变期。中位年龄为44岁（范围14至71岁），中位血小板计数为402×10⁹/L，中位白细胞计数为86×10⁹/L。17例慢性期早期CML患者中的14例接受了α干扰素治疗；12例（86%）实现了完全血液学缓解。慢性期CML的中位生存期为60个月（范围3至90多个月）。Ph阴性BCR阳性CML患者与Ph阳性CML患者具有相似的特征和结局。与Ph阴性BCR阴性CML和CMML患者相比，Ph阴性BCR阳性CML和Ph阳性CML患者明显更年轻，白细胞增多、血小板增多、外周血和骨髓嗜碱性粒细胞增多的发生率明显更高，贫血、血小板减少、骨髓原始细胞百分比以及外周血和骨髓单核细胞增多的发生率明显更低。Ph阴性BCR阳性CML的中位生存期为60个月，Ph阳性CML为73个月，Ph阴性BCR阴性CML为25个月，CMML为9个月（P<0.001）。在根据分期进行分析时，归类为Ph阴性BCR阳性CML的患者。I期疾病的生存期明显优于Ph阴性BCR阴性CML患者（P<0.02）。
Ph阴性BCR阳性CML患者与Ph阳性CML患者相似，应采用相同的治疗方法。
