髓细胞肉瘤临床病理特征并文献复习  

Clinic-pathological characteristics of myeloid sarcoma and review of literature

作　　者：舒汨汨 董宝侠 杨岚 白庆咸 陈协群 Shu Mimi;Dong Baoxia;Yang Lan;Bai Qingxian;Chen Xiequn(The Affiliated Hospital of Northwest University,Xi'an No.3 Hospital,Shaanxi Xi'an 710021,China;Department of Hematology,Xijing Hospital,the Air Force Military University,Shaanxi Xi'an 710032,China)

机构地区：[1]西北大学附属医院西安市第三医院,陕西西安710021 [2]空军军医大学西京医院血液科,陕西西安710032

出　　处：《现代肿瘤医学》2019年第24期4448-4451,共4页Journal of Modern Oncology

摘　　要：目的:探讨髓细胞肉瘤(myeloid sarcoma,MS)的临床表型、病理特征及治疗反应。方法:对西京医院2015年9月至2018年12月间收治的经组织病理学证实的7例MS的临床资料进行分析,并复习相关文献。结果:7例MS患者中位发病年龄40岁(22~62岁)。1例诊断MS时合并骨髓增生异常综合征(MDS),其余6例骨髓形态学检查正常。7例MS病变分别累及皮肤、淋巴结、胰腺、胆管、乳腺、胸壁及眼眶。6例患者接受联合化疗、去甲基化治疗或自体造血干细胞移植(ASCT)。中位生存期7个月。其中4例死亡患者中,1例患者诊断后8个月进展至急性髓系白血病(AML),经多疗程联合化疗后,生存期达31个月,3例未进展至AML的孤立性MS生存期仅5个月左右。至随访结束时,2例患者存活,疾病仍处于完全缓解状态。1例患者发病时伴发MDS,多疗程化疗方案含去甲基化药物;另1例患者接受大剂量化疗联合ASCT治疗。结论:进展至AML的MS,其临床预后可能不劣于孤立性MS;由去甲基化药物或ASCT组成的强化治疗方案似乎有益于改善MS的临床预后。Objective:To describe the clinical presentations,pathologic features and treatment response of myeloid sarcoma(MS).Methods:Seven MS patients pathologically diagnosed in Xi'jing Hospital from September 2015 to December 2018 were analysed.MS-associated literatures were reviewed.Results:Median age was 40 years(range:22~62).Bone marrow examination revealed a myelodysplastic condition in one patient and normal features in the other six patients.MS lesion involved skin,lymph nodes,pancreas,breast,bile duct,chest wall or orbital region.In the seven cases of MS patients,six patients received acute myeloid leukemia(AML)-like conventional or intensive treatments consisted of chemotherapy,hypomethylation,radiotherapy or autologous stem cell transplantation(ASCT).Median survival time from MS diagnosis was 7 months(range:5~31 months).Four of them died.A patient who presented isolated MS subsequently developed AML within 8 months from diagnosis,and obtained 31 months of survival after treated with polychemotherapy.By contrast,remaining three patients without evidence of AML on bone marrow examination only survived for about 5 months.Currently,two patients are still alive,for 17 months from diagnosis.One case with myelodysplastic condition received intensive treatments containing hypomethylating agent,and another was treated with high dose chemotherapy in combination with ASCT.Conclusion:Our data indicate that MS progressing to AML does not imply poorer prognosis compared with isolated MS,and intense treatments containing hypomethylating drug or ASCT appear to contribute to outcome of MS.

关 键 词：髓细胞肉瘤 化疗 去甲基化治疗 自体造血干细胞移植 

分 类 号：R733.3[医药卫生—肿瘤]