机构地区:[1]郑州大学第一附属医院儿童医院血液肿瘤科,450052
出 处:《国际儿科学杂志》2023年第6期403-409,共7页International Journal of Pediatrics
摘 要:目的探讨恶性实体瘤继发白血病患儿的临床特点、诊治方案及预后。方法选择2012年1月至2022年1月郑州大学第一附属医院收治的2275例15岁以下恶性实体瘤患儿中,随访至2022年8月1日,确诊为继发性白血病的6例患儿为研究对象,回顾性分析其临床资料,并进行文献复习。结果(1)共收治恶性实体瘤患儿2275例,男1369例,女906例;有6例患儿治疗后继发白血病,发生率0.26%,其中男4例,女2例;实体瘤发病年龄5.5(2.8,9.7)岁,继发白血病时年龄(9.1±3.9)岁,二者间隔时间(26.2±17.3)个月。(2)原发恶性实体瘤类型:按继发白血病时间依次为臀部恶性横纹肌样瘤1例、颅内髓母细胞瘤2例、颅内和盆腔恶性生殖细胞瘤各1例、胰母细胞瘤1例。1例患儿行神经内镜下脑室病损活检术,余5例均手术切除肿瘤;3例颅内肿瘤术后行局部瘤床+全脑全脊髓放疗;6例患儿均接受化疗,化疗药物主要有阿霉素、长春新碱、环磷酰胺、铂类、异环磷酰胺、依托泊苷、博来霉素、替莫唑胺等。3例获得完全缓解,1例部分缓解,1例疾病稳定,1例疾病进展。(3)继发白血病类型:急性髓系白血病(acute myeloid leukemia,AML)M53例、M1和M2各1例、急性B细胞白血病(acute B-lymphoblastic leukemia,B-ALL)1例。3例检测到融合基因阳性、基因突变及染色体核型异常。6例均拒行造血干细胞移植(hematopoietic stem cell transplantation,HSCT),1例B-ALL接受羟基脲及地塞米松治疗后放弃治疗,5例AML按中华医学会儿科学分会血液学组AML-2006方案化疗。2例早期死亡,4例在化疗1~2个疗程后获得完全缓解,其中2例因化疗过程中合并肺部感染、深部真菌病、骨髓炎等,3个疗程后未再继续治疗,后复发死亡;随访截止时,存活并继续治疗2例,其中1例复发。确诊继发性白血病后,6例患儿1年总生存率为(33±26)%。结论儿童恶性实体瘤继发白血病极少见,多为年长儿,发病与化疗或放疗�ObjectiveTo investigate the clinical characteristics,diagnosis and treatment and prognosis of children with leukemia secondary to malignant solid tumor.MethodsFrom January 2012 to January 2022,a total of 2275 children under 15 years of age with malignant solid tumor were admitted to the First Affiliated Hospital of Zhengzhou University.Six children diagnosed with secondary leukemia after follow-up to August 1,2022 were selected as the study objects.Their clinical data were retrospectively analyzed and the literature was reviewed.Results(1)A total of 2275 children with malignant solid tumors included 1369 males and 906 females.There were 6 children with secondary leukemia,with an incidence rate of 0.26%,including 4 males and 2 females.The age of onset of solid tumor was 5.5(2.8,9.7)years,and the age of secondary leukemia was(9.1±3.9)years,and the interval between them was(26.2±17.3)months.(2)Malignant solid tumor types:according to the time of secondary leukemia,there were hip malignant rhabdomyoma in 1 case,intracranial medulloblastoma in 2 cases,intracranial and pelvic malignant germinoma in 1 case respectively,and pancreatic blastoma in 1 case.Intracranial lesion biopsy was performed in 1 case,and tumor resection was performed in the other 5 cases.Three patients with intracranial tumors underwent local tumor bed,whole brain and spinal radiotherapy.All the 6 children received chemotherapy,and the main chemotherapy drugs were doxorubicin,vincristine,cyclophosphamide,platinum,ifosfamide,etoposide,bleomycin,temozolomide,etc.Complete remission was achieved in 3 cases,partial remission in 1 case,stable disease in 1 case,and disease progression in 1 case.(3)The secondary leukemia types were as follows:acute myeloid leukemia(AML)M5 in 3 cases,M1 in 1 case,M2 in 1 case,and acute B-lymphoblastic leukemia(B-ALL)in 1 case.All six cases refused hematopoietic stem cell transplantation(HSCT).One case with B-ALL gave up after receiving hydroxyurea and dexamethasone.Five cases with AML received chemotherapy according to the A
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