Hiorns L R, Swansbury G J, Mehta J, Min T, Dainton M G, Treleaven J, Powles R L, Catovsky D
Academic Department of Haematology and Cytogenetics, The Royal Marsden Hospital Trust, Sutton, Surrey.
Br J Haematol. 1997 Feb;96(2):314-21. doi: 10.1046/j.1365-2141.1997.d01-2037.x.
Acute promyelocytic leukaemia (APL) has been associated with a favourable prognosis in many studies of acute myeloid leukaemia. A series of 54 patients treated at the Royal Marsden Hospital between 1979 and 1996, with APL and the t(15;17) chromosome translocation at presentation, was examined for the effect of additional chromosome abnormalities in their presentation karyotype on survival. The patients were aged between 2 and 62 years with a median age of 31 years. There were approximately equal numbers of males and females. Presentation white cell count ranged from O.7 to 156 x 10(9)/l with a median of 1.0 x 10(9)/l. 39% of patients (21/54) had additional chromosome abnormalities at presentation. Statistical analyses were performed for factors thought to influence survival such as age, sex, white cell count, and number of courses of chemotherapy required to enter remission. These showed that the presence of additional chromosome abnormalities has an adverse effect on prognosis, independent of other prognostic indicators, reducing it to the level of patients with AML from less-favourable cytogenetic subgroups. These data indicate that additional therapeutic strategies may be required in patients with APL who demonstrate cytogenetic aberrations over and above the t(15;17) at presentation. The biological basis for the more aggressive nature of these cases remains to be determined.
在许多急性髓系白血病研究中，急性早幼粒细胞白血病（APL）的预后良好。对1979年至1996年间在皇家马斯登医院接受治疗的54例初诊时伴有t(15;17)染色体易位的APL患者进行了研究，以探讨其初诊核型中额外染色体异常对生存的影响。患者年龄在2岁至62岁之间，中位年龄为31岁。男女数量大致相等。初诊时白细胞计数范围为0.7至156×10⁹/L，中位值为1.0×10⁹/L。39%的患者（21/54）初诊时伴有额外染色体异常。对年龄、性别、白细胞计数以及达到缓解所需化疗疗程数等被认为影响生存的因素进行了统计分析。结果显示，额外染色体异常的存在对预后有不利影响，独立于其他预后指标，使其降至来自预后较差细胞遗传学亚组的急性髓系白血病患者的水平。这些数据表明，对于初诊时除t(15;17)外还存在细胞遗传学畸变的APL患者，可能需要额外的治疗策略。这些病例更具侵袭性的生物学基础仍有待确定。
