治疗相关性血液系统恶性疾病3例并文献复习

Treatment-related hematological malignant diseases:report of three cases and literature review

目的探讨儿童治疗相关性血液系统恶性疾病临床特点、高危因素、治疗方案及预后。方法回顾2019年5月以来收治的3例该类疾病患儿临床资料,并检索了中国知网、万方数据库中儿童此类疾病的病例报道,复习相关文献,并进行分析。结果(1)3例患儿在原发肿瘤确诊后均接受了化疗,在6~57个月后,例1、3出现治疗相关骨髓增生异常综合征(t-MDS),例2继发急性淋巴细胞白血病(t-ALL)。目前均存活,例1去甲基化治疗,部分缓解状态,例2接受造血干细胞移植(HSCT)后疾病缓解,例3给予对症支持治疗。(2)共检索到10篇文献,报道了16例患儿,男性12例,女性4例,起病年龄2~13岁,潜伏期16~74个月,15例为治疗相关髓系白血病(t-AML),1例为t-ALL,14例患儿有明确的烷化剂及拓扑异构酶抑制剂暴露,随访终点8例疾病进展死亡。结论治疗相关血液系统恶性疾病是一类由多因素共同作用的疾病类型。以t-AML多发,男性多于女性,既往治疗中使用烷化剂及拓扑异构酶抑制剂发生率明显增高,传统治疗预后差,死亡率高。减少化疗方案的重叠,采用新的基因靶向药物、去甲基化治疗、嵌合抗原受体T细胞治疗均可改善治疗效果,根治仍需HSCT。

Objective To explore the clinical characteristics,high-risk factors,treatment scheme and prognosis of treatment-related hematological malignancies in children.Methods Clinical data of 3 children with treatment-related hematological malignancies treated since May 2019 were analyzed retrospectively,and the case reports of children with such diseases in China knowledge network and Wanfang database were retrieved,the relevant literature was reviewed and analyzed.Results(1)Three children received chemotherapy after the diagnosis of primary tumor.After 6~57 months,cases 1 and 3 appeared treatment-related myelodysplastic syndrome(t-MDS)and case 2 was treatment-related acute lymphoblastic leukemia(T-ALL).At present,three children are all survived.Case 1 had a partial remission status after demethylation treatment.Case 2 experienced disease remission after receiving hematopoietic stem cell transplantation(HSCT).Case 3 received symptomatic support treatment.(2)A total of 10 literatures were retrieved,which reported 16 children had hematological malignant diseases after chemotherapy,including 12 males and 4 females,the onset age was 2~13 years old,the incubation period was 16~74 months;and 15 cases were treatment-related myeloid leukemia(t-AML),1 case was T-ALL;14 cases had definite exposure to alkylating agents and topoisomerase inhibitors;and 8 cases died of disease progression at the end of follow-up.Conclusions Treatment-related hematological malignant diseases are a type of disease affected by multiple factors.T-AML is more common,there are more male patients than female,and the incidence is significantly higher in patients previously treated with alkylating agents and topoisomerase inhibitors than that in patients with traditional treatment,the prognosis is poor and the mortality is high.Reducing the overlap of chemotherapy schemes,using new gene targeted drugs,demethylation therapy and chimeric antigen receptor T cells can improve the therapeutic effect,HSCT is still needed for radical cure.