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婴儿急性淋巴细胞白血病

Acute lymphoblastic leukemia in infancy.

作者信息

Silverman Lewis B

机构信息

Department of Pediatric Oncology, Dana-Farber Cancer Institute and Children's Hospital, Boston, Massachusetts 02115, USA.

出版信息

Pediatr Blood Cancer. 2007 Dec;49(7 Suppl):1070-3. doi: 10.1002/pbc.21352.

Abstract

Infant ALL is uncommon, biologically distinctive from the disease in older children, and associated with a relatively poor prognosis. Adverse prognostic factors include the presence of an MLL gene rearrangement (observed in up to 80% of infants with ALL), younger age at diagnosis, high presenting leukocyte counts, and slow early response to therapy. The role of stem cell transplant in first remission remains controversial. Current research efforts to improve the outcome of MLL-rearranged ALL in infants include clinical trials testing cytarabine-intensive regimens and translational investigations of novel, targeted therapies, such as FLT3-inhibitors.

摘要

婴儿急性淋巴细胞白血病(ALL)并不常见,在生物学上与大龄儿童的该疾病不同,且预后相对较差。不良预后因素包括存在MLL基因重排(在高达80%的婴儿ALL患者中观察到)、诊断时年龄较小、初诊时白细胞计数高以及对治疗的早期反应缓慢。干细胞移植在首次缓解中的作用仍存在争议。目前为改善婴儿MLL重排ALL的治疗结果所做的研究工作包括测试阿糖胞苷强化方案的临床试验以及新型靶向治疗(如FLT3抑制剂)的转化研究。

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