摘要
目的 观察干扰素-α、沙利度胺和羟基脲对真性红细胞增多症（PV）的临床疗效,并探讨其对JAK2-V617F基因突变的作用.方法 对确诊为PV且JAK2-V617F阳性、BCR-ABL阴性患者45例,分成羟基脲＋干扰素治疗组（A组）18例;羟基脲＋沙利度胺治疗组（B组）17例;对照组（C组）为因各种原因无法使用干扰素或沙利度胺而单用羟基脲治疗组10例.以2年为观察期,分别观察三组患者治疗前后白细胞计数、红细胞计数、血红蛋白量、红细胞压积、血小板计数、促红细胞生成素水平、JAK2-V617F表达、骨髓细胞形态学、骨髓病理学改变,及脾脏大小的改变等指标.以比较三组方案的疗效.结果 三组患者治疗前后,白细胞计数、红细胞计数、血红蛋白量、红细胞压积,血小板计数、促红细胞生成素水平和脾脏肿大程度均有改善,但三组疗效差异无统计学意义（P〉0.05）;A组和B组治疗后骨髓纤维化程度进展不明显,C组骨髓纤维化程度明显,差异存在统计学意义（P〈0.05）.三组治疗2年后JAK2-V617F基因突变均无转阴.结论 羟基脲联用干扰素或沙利度胺治疗真性红细胞增多症,与单用羟基脲相比,血液学指标改善疗效相似,对JAK2-V617F基因突变均无明显疗效,但在抑制骨髓纤维化方面疗效显著.
Objective To observe the efficacy of interferon-α, thalidomide and hydroxyurea on the treatment of polycythemia vera （PV） and JAK2-V617F gene mutation. Methods 45 cases diagnosed as PV with positive JAK2-V617F gene mutation and negative BCR-ABL gene mutation were divided into hydroxyurea plus interferon treatment group （group A, n=18）, hydroxyurea plus thalidomide treatment group （group B, n=17） and only hydroxyurea treatment control group （group C, n=10） and observed for 2 years. Serveral parameters such as patients＇ white blood cell count, red blood cell count, hemoglobin level, hematocrit level, platelet count, serum erythropoietin level, JAK2-V617F expression level, bone marrow morphology, bone marrow pathology and spleen size before and after treatment were recorded and compared among the three groups. Results After treatment, there was no significant difference in the parameters of white blood cell count, red blood cell count, hemoglobin concentration, hematocrit level, platelet count, serum erythropoietin level, spleen sizes of patients in all three groups （P〉0. 05）. The myelofibrosis degree of the patients from group A and group B did not progress markedly, but that of the patients from group C progressed significantly （P〈0. 05）. After 2 years of treatment, JAK2-V617F gene mutation is still positive in all three groups. Conclusion Combination of hydroxyurea with interferon or thalidomide was more effective in the treatment of PV. Although the combination therapy caused similar changes in hematologic parameters and JAK2-V617F gene mutation compared to hydroxyurea alone, it was more effective in the inhibition of myelofibrosis.
出处
《国际输血及血液学杂志》
CAS
2010年第4期299-302,共4页
International Journal of Blood Transfusion and Hematology
