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成人前体B细胞急性淋巴细胞白血病中存在两个以上重排的免疫球蛋白重链基因。

Presence of more than two rearranged immunoglobulin heavy-chain genes in adult precursor B-cell acute lymphoblastic leukemia.

作者信息

Schardt C, Hoelzer D, Ganser A

机构信息

Department of Hematology, University of Frankfurt/Main, Federal Republic of Germany.

出版信息

Ann Hematol. 1992 Feb;64(2):72-7. doi: 10.1007/BF01715348.

Abstract

We examined the configuration of the immunoglobulin genes in the leukemic blast cell DNA of 20 adults with precursor B-cell acute lymphoblastic leukemia (ALL), treated according to the BMFT protocol. Sixteen of 20 (80%) patients expressed HLA-DR antigens and lacked detectable T-cell antigens. Eleven of the 20 patients (55%) were positive for the CD10 antigen and therefore classified as common ALL. Six patients were classified by immunological phenotyping as null-ALL (30%). Three patients (15%) expressed both immature B-cell markers CD19, CD22, or CD24 and myelomonocytic markers CDw65 or CD15, suggesting precursor B-ALL with cross-lineage expression of myeloid markers. In 18 of the 20 patients (90%), rearrangements and/or deletions of the immunoglobulin heavy-chain (IgH) gene locus were found. In none of the patients was a light-chain gene rearrangement observed. Two patients (10%) had a rearrangement of one allele for the J beta 1 gene region of the TCR-beta gene. In four patients (20%) more than two hybridizing bands for the IgH genes were detected. Two of these four patients with multiple hybridizing bands for the IgH genes had a t (4;11) translocation. Two of five patients with the t (4;11) translocation co-expressed both B-cell antigens and the myeloid antigens CD15 or CDw65. No correlation was found between the immunophenotype of the ALL and the arrangement pattern of their IgH genes. Kaplan-Meier plot analysis revealed no significant difference between adult precursor B-ALL patients with monoclonal or oligoclonal IgH gene rearrangements and their disease-free survival rates.

摘要

我们检测了20例按照BMFT方案治疗的成年前体B细胞急性淋巴细胞白血病(ALL)患者白血病原始细胞DNA中免疫球蛋白基因的构型。20例患者中有16例(80%)表达HLA - DR抗原且未检测到T细胞抗原。20例患者中有11例(55%)CD10抗原呈阳性,因此被归类为普通ALL。6例患者经免疫表型分析归类为无标记ALL(30%)。3例患者(15%)同时表达未成熟B细胞标志物CD19、CD22或CD24以及髓单核细胞标志物CDw65或CD15,提示前体B - ALL伴有髓系标志物的跨系表达。20例患者中有18例(90%)发现免疫球蛋白重链(IgH)基因位点的重排和/或缺失。未观察到任何患者有轻链基因重排。2例患者(10%)TCR - β基因的Jβ1基因区域一个等位基因发生重排。4例患者(20%)检测到IgH基因有两条以上杂交带。这4例IgH基因有多个杂交带的患者中有2例发生了t(4;11)易位。5例发生t(4;11)易位的患者中有2例同时共表达B细胞抗原和髓系抗原CD15或CDw65。未发现ALL的免疫表型与其IgH基因排列模式之间存在相关性。Kaplan-Meier曲线分析显示,成年前体B - ALL患者单克隆或寡克隆IgH基因重排与其无病生存率之间无显著差异。

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