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费城染色体阳性成熟B细胞(伯基特细胞)白血病

Philadelphia chromosome-positive mature B-cell (Burkitt cell) leukaemia.

作者信息

Mann Georg, Trebo Monika M, Haas Oskar A, Grümayer-Panzer Eva R, Dworzak Michael N, Lion Thomas, Gadner Helmut

机构信息

Children's Cancer Research Institute, St. Anna Children's Hospital, Kinderspitalgasse 6, 1090 Vienna, Austria.

出版信息

Br J Haematol. 2002 Aug;118(2):559-62. doi: 10.1046/j.1365-2141.2001.03598.x.

DOI:10.1046/j.1365-2141.2001.03598.x
PMID:12139745
Abstract

Philadelphia chromosome-positive (Ph+) acute leukaemia usually shows lymphoblastic morphology and a B-precursor phenotype. The bone marrow aspirate of a 9-year-old boy showed a L3 blast cell morphology in 90% of cells; immunophenotyping revealed a mature B-blast population. The translocation t(9;22) (q34;q11) was seen in 45 out of 50 metaphases, and expression of the corresponding bcr1/abl fusion transcripts, but no IgH/myc co-localization or splitting of c-myc, was demonstrated. Chemotherapy according to the Berlin-Frankfurt-Munster non-Hodgkin's lymphoma (NHL-BFM 95) protocol with maintenance according to the BFM acute lymphoblastic leukaemia (ALL-BFM 90) protocol resulted in continuing complete remission of 54 months. The occurrence of Ph+ Burkitt's leukaemia might reflect multiple-step cancer development.

摘要

费城染色体阳性(Ph+)急性白血病通常表现为淋巴细胞形态和B前体表型。一名9岁男孩的骨髓穿刺显示90%的细胞为L3原始细胞形态;免疫表型分析显示为成熟B原始细胞群体。在50个中期细胞中有45个可见t(9;22)(q34;q11)易位,并且证实了相应bcr1/abl融合转录本的表达,但未发现IgH/myc共定位或c-myc分裂。按照柏林-法兰克福-明斯特非霍奇金淋巴瘤(NHL-BFM 95)方案进行化疗,并根据BFM急性淋巴细胞白血病(ALL-BFM 90)方案进行维持治疗,使完全缓解持续了54个月。Ph+伯基特白血病的发生可能反映了癌症的多步骤发展。

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引用本文的文献

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Precursor B-Cell Acute Lymphoblastic Leukemia/Lymphoma with L3 Morphology, Philadelphia Chromosome, MYC Gene Translocation, and Coexpression of TdT and Surface Light Chains: A Case Report.具有L3形态、费城染色体、MYC基因易位以及末端脱氧核苷酸转移酶(TdT)和表面轻链共表达的前体B细胞急性淋巴细胞白血病/淋巴瘤:一例报告
Case Rep Pathol. 2013;2013:679892. doi: 10.1155/2013/679892. Epub 2013 Mar 6.