摘要
目的总结先天性白血病的临床特点及预后,以提高对本病的认识。方法回顾性纳入2006年1月至2018年12月首都医科大学附属北京儿童医院诊治的10例先天性白血病患儿。总结患儿的临床表现、实验室检查、治疗及随访等相关临床资料。采用描述性统计分析。结果男婴6例,女婴4例。发病日龄均为生后11 d内。10例患儿的入院主诉主要是黄疸(n=4)、气促(n=3)、发热(n=2)和皮疹(n=2)等;体格检查有8例脾脏增大,7例肝脏增大,7例有皮肤出血点/皮疹。血常规检查白细胞计数均明显升高,为(45.8~553.0)×109/L,伴有程度不同的贫血及血小板减少。经骨髓穿刺诊断2例急性淋巴细胞白血病,8例急性髓细胞白血病。10例患儿均拒绝化疗。出院后3例失访;6例患儿自动出院后2个月内死亡;1例获自然缓解但2年后复发,经规律治疗后获完全缓解,随访至10岁未复发。结论先天性白血病病情重,病死率高。部分病例可获自然缓解,但须长期随访。
Objective To have a better understanding of congenital leukemia by summarizing its clinical features and prognosis.Methods This study retrospectively recruited 10 neonates with congenital leukemia treated in Beijing Children's Hospital,Capital Medical University from January 2006 to December 2018.Clinical data including clinical manifestations,laboratory examinations,treatment and follow-up were described.Results The 10 patients consisted of six boys and four girls.In all cases,symptoms presented within 11 d after birth.The admission complaints were jaundice(n=4),polypnea(n=3),fever(n=2)and rash(n=2).Physical examinations of the 10 patients showed eight with splenomegaly,seven with hepatomegaly and seven with petechia/skin rash.All patients had significantly increased white blood cell count(from 45.8×109/L to 553.0×109/L),complicated by different degrees of anemia and thrombocytopenia.By bone marrow biopsy,two cases were diagnosed as acute lymphoblastic leukemia and the other eight cases as acute myeloid leukemia.All cases refused chemotherapy on the preliminary diagnosis.Three cases lost follow-up and six died within two months after discharge requested by their parents.One baby had spontaneous remission,but relapsed two years later.Complete remission was achieved after strict management and no relapse was reported until ten years old.Conclusions Congenital leukemia is a severe condition with high mortality.Some cases may achieve spontaneous remission,but long-term follow-up is needed.
作者
杨学芳
罗洁
王亚娟
张瑞东
邵芳
姜敏
王慧欣
Yang Xuefang;Luo Jie;Wang Yajuan;Zhang Ruidong;Shao Fang;Jiang Min;Wang Huixin(Department of Comprehensive Ward,Beijing Children’s Hospital,Capital Medical University,National Center for Children's Health,Beijing 100045,China;Department of Neonatology,Beijing Children’s Hospital,Capital Medical University,National Center for Children's Health,Beijing 100045,China;Department of Hematology Center,Beijing Children’s Hospital,Capital Medical University,National Center for Children's Health,Beijing 100045,China)
出处
《中华围产医学杂志》
CAS
CSCD
北大核心
2020年第7期469-475,共7页
Chinese Journal of Perinatal Medicine
关键词
白血病
随访研究
先天性
Leukemia
Follow-up studies
Congenital
