原发性骨髓纤维化转化为急性B淋巴细胞白血病1例并文献复习  

Transformation of primary myelofibrosis to B-cell acute lymphoblastic leukemia: report of 1 case and review of literature

作　　者：丁冰洁 刘柳[2] 李梦娟 夏傲 宋雪雯 徐佩佩 刘建平 周虎 宋永平 Ding Bingjie;Liu Liu;Li Mengjuan;Xia Ao;Song Xuewen;Xu Peipei;Liu Jianping;Zhou Hu;Song Yongping(Department of Hematology,Afiliated Cancer Hospital of Zhengzhou University,Henan Cancer Hospital,Zhengzhou 450008,China;Department of Hematology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou450052,China)

机构地区：[1]河南省肿瘤医院,郑州大学附属肿瘤医院血液科,郑州450008 [2]郑州大学第一附属医院血液科,郑州450052

出　　处：《白血病．淋巴瘤》2022年第10期606-609,共4页Journal of Leukemia & Lymphoma

基　　金：国家自然科学基金(82070120,81370615、81600097)。

摘　　要：目的:探讨原发性骨髓纤维化(PMF)转化为急性B淋巴细胞白血病(B-ALL)的发病机制及预后。方法:回顾性分析2018年11月郑州大学附属肿瘤医院收治的1例PMF转化为B-ALL患者的诊断及治疗过程,并复习相关文献。结果:患者,女性,64岁,初始诊断为PMF,先后使用泼尼松、达那唑、左旋咪唑、阿司匹林、沙利度胺及杰克替尼等治疗。17个月后复查示转化为B-ALL,给予诱导治疗,持续缓解8个月后复查示ALL复发。再诱导治疗后未缓解,2个月后放弃治疗出院。转化为白血病前后JAK2 V617F基因突变均为阳性。结论:PMF转化为B-ALL的患者临床预后差,生存时间短。其原因可能与额外的遗传学事件或某些高风险基因相关,但具体转化机制仍不清楚,需要进一步探究病因,寻求针对性治疗方法。Objective:To investigate the pathogenesis and prognosis of transformation of primary myelofibrosis (PMF) to B-cell acute lymphoblastic leukemia (B-ALL).Methods:The diagnosis and treatment process of a patient transferred from PMF to B-ALL in Affiliated Tumor Hospital of Zhengzhou University in November 2018 were retrospectively analyzed, and the relevant literature was reviewed.Results:The patient was a 64-year-old female, she was initially diagnosed with PMF, and then she developed B-ALL 17 months later after receiving treatment of prednisone, danazole, levamisole, aspirin, thalidomide and jaktinib. After induction therapy, the patient received 8 months of continuous remission, and then the reexamination showed relapse. There was no remission after reinduction therapy. The patient gave up treatment and was discharged 2 months later. JAK2 V617F gene mutation was positive before and after leukemia transformation.Conclusions:The patients with transformation of PMF to B-ALL have poor clinical prognosis and short survival time. The possible mechanism of its transformation may be related to additional genetic events or certain high-risk genes. However, the specific mechanism is still unclear, and further investigation of the etiology is needed to seek targeted treatment.

关 键 词：原发性骨髓纤维化急性 淋巴细胞白血病 发病机制 

分 类 号：R733.71[医药卫生—肿瘤]