Griesshammer M, Heinze B, Hellmann A, Popp C, Anger B, Heil G, Bangerter M, Heimpel H
Department of Clinical Physiology, University of Ulm, Germany.
Ann Hematol. 1996 Nov;73(5):225-30. doi: 10.1007/s002770050233.
Ninety patients with Philadelphia chromosome-positive chronic myelogenous leukemia in blast crisis were reviewed to identify significant prognostic associations. At diagnosis of blast crisis the main clinical, laboratory, and cytogenetic data were recorded and evaluated for prognostic significance. At the time of the analysis 89 patients had died, with a median survival of 11 weeks from diagnosis of blast crisis. Patient characteristics demonstrated in the univariate analysis to have significant association with shorter survival were: thrombocythemia, leukocyte count above 20 x 10(9), Karnofsky index < 50%, nonlymphoid blast cell morphology, cytogenetic clonal evolution, the presence of a double Philadelphia chromosome or trisomy 8, and no response to therapy. In 17 of 59 patients (29%) evaluable for response to therapy a complete or partial remission was achieved. These responders had a significantly longer median survival (25 weeks) as compared with nonresponders (9 weeks). Response to therapy was significantly better in lymphoid blast crisis and in patients without clonal evolution. In a multivariate analysis containing all significant variables of the univariate analysis two parameters retained their prognostic significance: response to therapy and trisomy 8. In spite of the short overall survival in blast crisis, the determination of prognostic factors may be a useful tool for the clinician planning therapy, especially new therapeutic approaches.
回顾了90例费城染色体阳性的慢性粒细胞白血病急变期患者，以确定显著的预后相关性。在急变期诊断时，记录主要的临床、实验室和细胞遗传学数据，并评估其预后意义。在分析时，89例患者已经死亡，从急变期诊断起的中位生存期为11周。单因素分析显示与较短生存期有显著相关性的患者特征为：血小板增多、白细胞计数高于20×10⁹、卡氏评分<50%、非淋巴细胞母细胞形态、细胞遗传学克隆演变、双费城染色体或8号染色体三体的存在以及对治疗无反应。在59例可评估治疗反应的患者中，17例（29%）获得了完全或部分缓解。与无反应者（9周）相比，这些有反应者的中位生存期显著更长（25周）。在淋巴细胞母细胞危象和无克隆演变的患者中，对治疗的反应明显更好。在包含单因素分析所有显著变量的多因素分析中，两个参数保留了其预后意义：对治疗的反应和8号染色体三体。尽管急变期的总体生存期较短，但确定预后因素可能是临床医生规划治疗，尤其是新治疗方法的有用工具。
