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成人t(4;11)(q21;q23)/MLL-AF4阳性B细胞急性淋巴细胞白血病的发病机制、临床及预后特征

Pathogenetic, Clinical, and Prognostic Features of Adult t(4;11)(q21;q23)/MLL-AF4 Positive B-Cell Acute Lymphoblastic Leukemia.

作者信息

Marchesi F, Girardi K, Avvisati G

机构信息

Unit of Hematology, Stem Cell Transplantation, Transfusion Medicine and Cellular Therapy, Campus Bio-Medico University Hospital, Via Àlvaro del Portillo 200, 00128 Rome, Italy.

出版信息

Adv Hematol. 2011;2011:621627. doi: 10.1155/2011/621627. Epub 2011 Nov 15.

Abstract

Translocation t(4;11)(q21;q23) leading to formation of MLL-AF4 fusion gene is found in about 10% of newly diagnosed B-cell acute lymphoblastic leukemia (ALL) in adult patients. Patients expressing this chromosomal aberration present typical biological, immunophenotypic, and clinical features. This form of leukemia is universally recognized as high-risk leukemia and treatment intensification with allogeneic hematopoietic stem cell transplantation (HSCT) in first complete remission (CR) could be a valid option to improve prognosis, but data obtained from the literature are controversial. In this review, we briefly describe pathogenetic, clinical, and prognostic characteristics of adult t(4;11)(q21;q23)/MLL-AF4 positive ALL and provide a review of the clinical outcome reported by the most important cooperative groups worldwide.

摘要

在成年患者新诊断的B细胞急性淋巴细胞白血病(ALL)中,约10%可发现导致MLL-AF4融合基因形成的t(4;11)(q21;q23)易位。表达这种染色体畸变的患者具有典型的生物学、免疫表型和临床特征。这种白血病形式被公认为高危白血病,首次完全缓解(CR)时采用异基因造血干细胞移植(HSCT)强化治疗可能是改善预后的有效选择,但从文献中获得的数据存在争议。在本综述中,我们简要描述成年t(4;11)(q21;q23)/MLL-AF4阳性ALL的发病机制、临床和预后特征,并对全球最重要的合作组报告的临床结果进行综述。

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