小儿髓系白血病的髓外受累:诊断和治疗的挑战。临床病例及文献复习

Q4 Medicine
Y. Dinikina, A. Maschan
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引用次数: 0

摘要

儿童急性髓性白血病(AML)的髓外(EM)累及问题具有相当大的相关性,因为其发病机制尚未得到充分研究,对预后的影响仍不清楚。组织和器官受累的变异性取决于骨髓细胞的免疫表型、细胞遗传学和分子特征,并可能导致诊断困难,因此有必要将影像学和实验室工具相结合,以及时准确地诊断EM疾病。EM的预后意义尚未明确确定,因此是否需要加强化疗,以及在首次缓解时进行异体造血干细胞移植,仍然存在争议。靶向治疗EM AML的结果令人鼓舞,并可能降低AML复发的风险。本文描述了儿童EM AML的临床特征,综述了诊断方法以及现有实验室和影像学方法的优点和局限性。本文还分析了EM AML的分子特征、目前的治疗方案和预后。患者的父母同意使用他们孩子的数据,包括照片,用于研究目的和出版物。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Extramedullary involvement in pediatric myeloid leukemia: challenges of diagnosis and treatment. Clinical cases and a literature review
   The problem of extramedullary (EM) involvement in acute myeloid leukemia (AML) in children is of considerable relevance since its pathogenesis remains understudied and the impact on prognosis is still unclear. The variability of tissue and organ involvement depends on immunophenotypic, cytogenetic, and molecular features of myeloid cells and can cause difficulties in diagnosis, thus making it necessary to combine imaging and laboratory tools for timely and accurate diagnosis of EM disease. The prognostic significance of EM involvement has not been established unequivocally, thus the need for intensification of chemotherapy, as well as for allogeneic hematopoietic stem cell transplantation in first remission, remain debatable. The results of target therapy in EM AML are encouraging and may reduce the risk of AML relapse. This article describes the clinical features of EM AML in children and reviews the diagnostic approaches as well as the advantages and limitations of existing laboratory and imaging methods. The molecular features of EM AML, current treatment options and prognosis have also been analyzed. The patients' parents gave their consent to the use of their children's data, including photographs, for research purposes and in publications.
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来源期刊
Pediatric Hematology/Oncology and Immunopathology
Pediatric Hematology/Oncology and Immunopathology Medicine-Pediatrics, Perinatology and Child Health
CiteScore
0.40
自引率
0.00%
发文量
49
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