以髓系肉瘤为首发表现的儿童急性髓系白血病临床特点  

Clinical characteristics of childhood acute myeloid leukemia with myeloid sarcoma as the first manifestation

作　　者：郭丽丽 刘炜 马平 宋丽丽 管玉洁 林巍[2] GUO Lili;LIU Wei;MA Ping;SONG Lili;GUAN Yujie;LIN Wei(Departmentof Hematology and Oncology,Children′s Hospital Affiliated to Zhengzhou University,Henan Children′s Hospital,Zhengzhou 450018,China;Leukemia Department,Beijing Children′s Hospital,Capital Medical University,National Centerfor Children′s Health,Beijing 100045,China)

机构地区：[1]河南省儿童医院郑州儿童医院血液肿瘤科,郑州450018 [2]国家儿童医学中心、首都医科大学附属北京儿童医院白血病科,北京100045

出　　处：《中国小儿血液与肿瘤杂志》2024年第6期431-434,共4页Journal of China Pediatric Blood and Cancer

摘　　要：目的探讨儿童以髓系肉瘤为首发表现的急性髓系白血病的特点,以加强对疾病认识。方法回顾性分析我院5例以髓系肉瘤起病的急性髓系白血病患儿的临床资料、治疗及预后。结果5例患儿中男3例,女2例,2例以腰背部疼痛,2例以腹痛、腹胀,1例以皮肤肿物为主要症状入院,起病部位椎管、阑尾各2例,皮肤1例;5例患儿均存在正细胞性贫血;病理免疫组化均表达MPO、CD43;骨髓检查M23例,M41例,M51例,AML1-ETO基因2例,KMT2A-MLLT10基因1例,CBFβ-MYH11伴kit突变1例。1例确诊后未治疗死亡,1例化疗后未行造血干细胞移植,复发死亡,3例化疗后行造血干细胞移植,随访至2024年1月均无病存活。结论儿童以髓系肉瘤起病的急性髓系白血病起病隐匿,临床表现异质性强,化疗虽敏感,长期预后差,需尽早行造血干细胞移植治疗。Objective To investigate the attributes of acute myeloid leukemia in children with myeloid sarcoma as the initial clinical manifestation,with the aim of improving comprehension of the disease.Methods We conducted a retrospective analysis of the clinical data,therapy,and prognosis of 5 children with myeloid sarcoma as their initial clinical manifestation at our hospital.Results Out of the 5 cases involving children,three were males and two were females.Among them,2 cases were admitted with low back pain,2 cases with abdominal pain and distension,1 case with skin mass as the main symptom.There were 2 cases in spinal canal,2 cases in appendix and 1 case in skin.All 5 cases had positive cell anemia.The pathological immunohistochemicals of myelosarcoma all express MPO and CD43.Bone marrow examination included 3 cases of M2,1 case of M4,1 case of M5.2 cases with the AML1-ETO fusion gene,1 case with the KMT2A-MLLT10 fusion gene,and 1 case with the CBFβ-MYH11 fusion gene along with a kit mutation.One patient died without treatment after diagnosis,1 patient did not undergo hematopoietic stem cell transplantation after chemotherapy and died of relapse.Three patients underwent hematopoietic stem cell transplantation after chemotherapy,and none of them survived until January 2024.Conclusions The onset of acute myeloid leukemia in children with myeloid sarcoma is insidious,the clinical manifestations are heterogeneous,although patients are sensitive to chemotherapy,the long-term prognosis is poor,hematopoietic stem cell transplantation is necessary as early as possible.

关 键 词：儿童 髓系肉瘤 急性髓系白血病 造血干细胞移植 

分 类 号：R73[医药卫生—肿瘤]