摘要
Abstract
Primary myelofibrosis(PMF) is a chronic clonal myeloid disease,which is classified as BCR-ABL1-negative myeloproliferative neoplasms(MPN) by WHO.The diagnostic criteria is updated in 2016,which presents the concept ofpre PMF and overt PMF,and further identifies PMF and essential thrombocytosis(ET).Patients often have physical symptoms,cachexia,anemia,splenomegaly,extramedullary hematopoiesis (EMH),JAK2,CALR or MPL mutation,abnormal cytokine expression,bone marrow fibrosis and shortened survival,and may advance to leukemia.The only radical cure is allogeneic hematopoietic stem cell transplantation (allo-HSCT),and other methods include drug therapy,splenectomy,and involved-field radiotherapy.In recent years,the presence ofJAK inhibitors significantly prolongs overall survival of patients.关键词
原发性骨髓纤维化/纤维化早期/纤维化明显期/JAK抑制剂Key words
primary myelofibrosis/pre primary myelofibrosispre/overt primary myelofibrosispre/JAK inhibitors分类
医药卫生
