Lin Yan, Lin Rongrong, Zhou Guangquan, Liu Yue, Dong Weimin, Cao Yang, Xie Xiaobao, Gu Weiying
Department of Haematology, The First People's Hospital of Changzhou, Third Affiliated Hospital of Soochow University, Changzhou, China.
J Int Med Res. 2019 Feb;47(2):1064-1071. doi: 10.1177/0300060518820147. Epub 2019 Jan 7.
Primary myelofibrosis (PMF) is a type of cloned myeloproliferative neoplasm stemming from haematopoietic stem cells, and tends to transform to acute myeloid leukaemia (AML) in approximately 10-20% of cases over a 10-year period. The transformation into AML has a poor prognosis, with a median overall survival of only 2.6 months in patients receiving supportive treatment. To date, treatment of AML transformation remains poor. The case of a 58-year-old female patient with AML transformed from PMF, who was treated with decitabine combined with all-trans retinoic acid, is reported. The patient had complete remission and a 17-month overall survival from initial diagnosis of transformed AML, with tolerated haematologic toxicity during the treatment period.
原发性骨髓纤维化（PMF）是一种起源于造血干细胞的克隆性骨髓增殖性肿瘤，在10年期间约10%-20%的病例中倾向于转化为急性髓系白血病（AML）。转化为AML的预后较差，接受支持性治疗的患者总中位生存期仅为2.6个月。迄今为止，AML转化的治疗效果仍然不佳。本文报告了一例58岁女性患者，其AML由PMF转化而来，接受了地西他滨联合全反式维甲酸治疗。该患者获得完全缓解，自转化型AML初始诊断起总生存期为17个月，治疗期间血液学毒性可耐受。
