伊马替尼治疗bcr-abl阳性原发性血小板增多症一例并文献复习

目的 观察伊马替尼治疗bcr-abl融合基因阳性原发性血小板增多症(ET)的疗效.方法 以伊马替尼(200～400mg/d)治疗1例对羟基脲(HU)产生耐药的bcr-abl阳性ET,并复习有关文献.结果 1例bcr-abl阳性ET初始接受HU 1.5～2.0 g/d,血小板降至562×109/L;16个月后,上述剂量的HU已难以使血小板降至1000×109/L以下(1050～1330)×1 09/L;HU加量至3.0 g/d,患者白细胞降至(0.3～0.9)×109/L,而血小板为(1290～1780)×109/L;换用伊马替尼(400 mg/d)治疗1个月,患者血小板390×109/L,白细胞0.5×109/L;将伊马替尼降至200～300 mg/d,1个月后患者白细胞与血小板维持在正常水平,2个月后bcr-abl融合基因转为阴性.结论 伊马替尼可能是治疗bcr-abl阳性ET的有效靶向药物,并且bcr-abl阳性ET对低剂量伊马替尼亦是敏感的.

Bcr-abl gene positive essential thrombocythemia treated with imatinib: one case and literatures review

Objective To observe the efficacy of imatinib on the treatment of bcr-abl positive essential thrombocythemia (ET). Methods A case of bcr-abl positive ET resistant to hydroxyurea (HU) treating with imatinib (200～400 mg/d) was reported and related literatures were reviewed. Results A case of bcr-abl positive ET was initially treated with 1.5～2.0 g/d HU, the platelet count decreased to 562x109/L after 4 weeks; however, the platelet count increased to (1020～1330)×109/L treating with same dose of HU 16 months later. With the elevation of HU to 3.0 g/d, platelet count was still high(1290～1780)x109/L companied with the very low white blood cell count(0.3～0.9)×109/L. While treating with imatinib (400 mg/d) for 1 month,the platelet count decreased to 390×109/L and white blood cell count was 0.5×109/L; Furthermore, treating with 200×300 mg/d of imatinib, the platelet and white blood cell count recovered in normal after 1 month,and bcr-abl fusion gene negative 2 months later. Conclusion Imatinib may be the effective targeting drug for the bcr-abl positive ET, and the bcr-abl positive ET is sensitive to low dose imatinib.