Kantarjian Hagop M, Bueso-Ramos Carlos E, Talpaz Moshe, O'Brien Susan, Giles Francis, Faderl Stefan, Wierda William, Rios Mary Beth, Shan Jianqin, Cortes Jorge
Department of Leukemia, The University of Texas M. D. Anderson Cancer Center, Houston, Texas 77030, USA.
Cancer. 2005 Aug 15;104(4):777-80. doi: 10.1002/cncr.21235.
Myelofibrosis is associated historically with a poor prognosis in patients with chronic myelogenous leukemia (CML). Its significance in the recent era of effective therapy with imatinib mesylate is unknown.
The current study evaluated the significance of the degree of pretreatment myelofibrosis on response and survival with imatinib therapy in patients with newly diagnosed CML. The study group comprised 198 patients with newly diagnosed Philadelphia chromosome-positive, chronic-phase CML treated with imatinib mesylate therapy. They were analyzed for the prognostic significance of bone marrow reticulin fibrosis.
Severe reticulin (Grade 3-4) fibrosis was observed in 75 patients (38%): Grade 3 in 46 (23%) patients and Grade 4 in 29 (15%) patients. There was a trend towards a lower incidence of a complete cytogenetic response in patients with Grade 4 reticulin fibrosis (76% vs. 89%; P = 0.07), and a significantly worse survival (estimated 3-year survival rate of 87% vs. 97%; P = 0.04).
Although the prognostic significance of severe reticulin fibrosis in patients with newly diagnosed CML receiving imatinib therapy was better, 15% of patients with Grade 4 reticulin fibrosis still had a worse outcome.
从历史上看，骨髓纤维化与慢性粒细胞白血病（CML）患者的不良预后相关。在最近使用甲磺酸伊马替尼进行有效治疗的时代，其意义尚不清楚。
本研究评估了新诊断的CML患者预处理时骨髓纤维化程度对伊马替尼治疗反应和生存的意义。研究组包括198例新诊断的费城染色体阳性、慢性期CML患者，接受甲磺酸伊马替尼治疗。分析了骨髓网硬蛋白纤维化的预后意义。
75例患者（38%）观察到严重的网硬蛋白（3-4级）纤维化：46例（23%）患者为3级，29例（15%）患者为4级。4级网硬蛋白纤维化患者完全细胞遗传学反应的发生率有降低趋势（76%对89%；P = 0.07），生存情况明显更差（估计3年生存率87%对97%；P = 0.04）。
尽管新诊断的接受伊马替尼治疗的CML患者中严重网硬蛋白纤维化的预后意义较好，但15%的4级网硬蛋白纤维化患者结局仍然较差。
