慢性粒细胞白血病继发性骨髓纤维化71例诊治体会

  目的  分析慢性粒细胞白血病(CML)合并骨髓纤维化(MF)患者的临床治疗效果及随访结果，为临床提供治疗及预后的相关数据。  方法  回顾性分析2014年1月—2019年6月衢州市人民医院血液科收治的71例CML合并MF患者的临床资料。CML病程3~9年，均在医院接受了系统治疗。在治疗维持或缓解期出现了进行性贫血症状、广泛性骨痛、巨脾等，符合MF(Ⅰ度)的诊断。CML患者采用格列卫治疗:餐前口服，1次/d，400~600 mg/次；或2次/d，800 mg/次；持续4周为1个疗程，共治疗4个疗程；CML合并MF患者采用芦可替尼治疗:基线血小板(PLT)在(100~200)×109/L时，起始剂量为2次/d，15 mg/次；基线PLT>200×109/L时，起始剂量为2次/d，20 mg/次。观察患者治疗效果及生存情况。  结果  治疗1周后患者疼痛、贫血症状有改善，可平卧, 脾在肋下 < 8 cm；治疗5个月后患者骨髓部分恢复造血功能，分类细胞形态大致正常，Hb≥100 g/L，WBC为(9~10)×109/L，脾在肋下 < 7 cm。治疗期间患者并发症发生率为15.49%(11/71)。平均随访时间为12~63个月，9例失访(占12.68%)。19例(26.76%)存活且无复发；38例(53.52%)存活但病情复发，转为急变期，中位转为急变时间为35.8个月；5例(7.04%)因合并重症感染(2例中枢神经系统感染，3例呼吸道感染)而死亡。  结论  靶向药物疗法对于CML继发MF患者的治疗可以取得一定的近期疗效，病情转为急变期及生存与否等远期疗效与发生骨髓纤维化的病变情况程度有关，骨髓纤维化加重可促使病情突变或死亡。 

Diagnosis and treatment of 71 cases of chronic myeloid leukaemia with secondary myelofibrosis

Objective To analyse the clinical treatment effect and follow-up results of patients with chronic myeloid leukaemia(CML)complicated with myelofibrosis(MF)and to provide relevant data for clinical treatment and prognosis.Methods The clinical data of 71 patients with CML complicated with MF admitted in the Department of Hematology of Quzhou People’s Hospital from January 2014 to June 2019 were analysed retrospectively.The course of CML was 3-9 years,and all of the patients received systematic treatment in the hospital.In the treatment maintenance or remission period,progressive anaemia,generalised bone pain and megaspleen appeared,which was consistent with the diagnosis of MF(I degree).CML:Glivec(400-600 mg/time,1 time/day;or 800 mg/time,2 times/day,800 mg/time)was used in the treatment for 4 weeks/course×4 courses;CML secondary bone fibrosis:when the baseline PLT was 100-200×10⁹/L,the initial dose of lucotinib was 2 times/day,15 mg/time;when the baseline PLT was>200×10⁹/L,the initial dose was 2 times/day,20 mg/time.The treatment effect,follow-up results and survival rate were observed.Results Clinical treatment results:after 1 week of treatment,the symptoms of pain and anaemia were improved,and the spleen was less than 8 cm under the rib;after 5 months of treatment,the haematopoietic function of the bone marrow was partially restored,and the morphology of classified cells was generally normal;haemoglobin level was≥100 g/L;white blood cell count was 9-10×10⁹/L,and the spleen was<7 cm under the rib.The incidence of complications was 15.49%(11/71).The average follow-up time was 12-63 months.Follow-up results:9 cases were lost(12.68%);19 cases survived without recurrence(26.76%);38 cases(53.52%)survived but relapsed and turned into cataclysm.The median time of transformation was 35.8 months.Five cases(7.04%)died of severe infection(two cases of central nervous system infection and three cases of respiratory tract infection).Conclusion Targeted drug therapy can achieve a certain short-term effect in the treatment of patients with MF secondary to CML.The long-term effects such as the change of the condition to cataclysm and survival are related to the severity of myelofibrosis.The aggravation of bone marrow fibrosis can promote the change or death of the disease.