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一种成熟B细胞白血病与4p+染色体异常的关联:一个细胞系的建立与特性分析

Association of a mature B cell leukemia with a 4p+ chromosomal abnormality: derivation and characterization of a cell line.

作者信息

Gribbin T E, Stein C K, Harrison J S, Glover T W, Hanson C A, Wasmuth J J, Cody R L, Mitchell B S

机构信息

Department of Internal Medicine, University of Michigan, Ann Arbor 48109.

出版信息

Leukemia. 1989 Sep;3(9):643-7.

PMID:2548046
Abstract

We have found a single 4p+ chromosomal abnormality, 46,XX, -4, +der(4)t(3;4)(q13.3;p16), in a patient with an unusual B cell leukemia of mature phenotype characterized by a high white cell count, tartrate-resistant acid phosphatase-positive malignant cells, splenic white pulp proliferation, and a serum IgM monoclonal gammopathy. The malignant cells were characterized by surface expression of CD19 (B4), CD20 (B1), IgM, IgD, kappa, and HLA-DR. They were weakly positive for CD21 (B2) and negative for CD25 (interleukin-2 receptor). The malignant cells also showed clonal rearrangement of the immunoglobulin heavy chain and kappa light chain genes. A cell line, designated HCLW-3B, was derived from unstimulated peripheral blood obtained during the leukemic phase and was found to contain the same 4p+ chromosomal abnormality as well as genomic sequences of the Epstein-Barr virus nuclear antigen. A somatic cell hybrid constructed from HCLW-3B containing the derivative chromosome 4 was used to confirm that chromosome 3q was the source of the translocated material. The availability of a cell line which is clonally derived from the patient's circulating leukemia cells should permit further characterization of this translocation at the molecular level.

摘要

我们在一名患有成熟表型的不寻常B细胞白血病患者中发现了一种单一的4p+染色体异常,核型为46,XX, -4, +der(4)t(3;4)(q13.3;p16),该患者的特征为白细胞计数高、抗酒石酸酸性磷酸酶阳性的恶性细胞、脾白髓增殖以及血清IgM单克隆丙种球蛋白病。恶性细胞的特征为表面表达CD19(B4)、CD20(B1)、IgM、IgD、κ以及HLA-DR。它们CD21(B2)弱阳性,CD25(白细胞介素-2受体)阴性。恶性细胞还显示免疫球蛋白重链和κ轻链基因的克隆性重排。一个名为HCLW-3B的细胞系源自白血病期获取的未受刺激的外周血,发现其含有相同的4p+染色体异常以及爱泼斯坦-巴尔病毒核抗原的基因组序列。由含有衍生4号染色体的HCLW-3B构建的体细胞杂种用于确认3q染色体是易位物质的来源。一个源自患者循环白血病细胞的克隆细胞系的存在应能使我们在分子水平上进一步鉴定这种易位。

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