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建立并鉴定源自一名骨髓增生异常综合征患者的B细胞系,该细胞系表达髓单核细胞和淋巴细胞标志物。

Establishment and characterization of a B-cell line derived from a patient with a myelodysplastic syndrome which expresses myelomonocytic and lymphoid markers.

作者信息

Bergamaschi G, Stella C C, Cattoretti G, Invernizzi R, Maserati E, Nalli G, Pedrazzoli P, Peverali F A, Della Valle G, Ascari E

机构信息

Dipartimento di Medicina Interna e Terapia Medica, University of Pavia, Italy.

出版信息

Br J Haematol. 1991 Jun;78(2):167-72. doi: 10.1111/j.1365-2141.1991.tb04412.x.

Abstract

We describe a novel continuous B-cell line (PV-90) derived from a patient with myelodysplastic syndrome (MDS) and originating from spontaneous infection with the Epstein-Barr virus (EBV). The patient progressed to acute myeloblastic leukaemia (AML) 5 months after clinical onset of MDS. PV-90 is of clonal origin as indicated by the presence of immunoglobulin (Ig) gene rearrangements, monoclonal surface immunoglobulins, and a single DNA restriction fragment corresponding to the EBV genomic termini. PV-90 cells also express a number of myelomonocytic markers, including alpha-naphthyl acetate esterase (ANAE), coagulation factor XIII, and CD68 antigen. Moreover, PV-90 cells constitutively express the c-fms proto-oncogene mRNA as the patient's blast cells did. Whereas a trisomy 11 (+11) was found in the patient's bone marrow cells, PV-90 cells had a normal karyotype initially, but at 4 months showed two different and independent chromosomal abnormalities: 90, XX, -Y, -Y, t(9;16) (q11;p13), and 90, XX, -Y, -Y, t(17;18) (p13;q21), the latter possibly involving the p53 (17,p13) and bcl-2 (18, q21) proto-oncogenes. The early development of these chromosomal aberrations is consistent with a genetic instability of PV-90 cells. Expression of bi-lineage markers and genetic instability may suggest that PV-90 cells originated from transformation of a myelodysplastic progenitor cell capable of both myeloid and B-cell differentiation. The PV-90 cell line might be useful in a number of studies, including the possible role of c-fms in cell differentiation, pathogenetic mechanisms of human preleukaemia and lineage promiscuity in acute leukaemia.

摘要

我们描述了一种新的连续B细胞系(PV-90),它源自一名骨髓增生异常综合征(MDS)患者,由爱泼斯坦-巴尔病毒(EBV)自发感染所致。该患者在MDS临床发病5个月后进展为急性髓细胞白血病(AML)。PV-90具有克隆起源,这可通过免疫球蛋白(Ig)基因重排、单克隆表面免疫球蛋白以及与EBV基因组末端相对应的单个DNA限制性片段的存在来表明。PV-90细胞还表达多种髓单核细胞标志物,包括α-萘乙酸酯酶(ANAE)、凝血因子XIII和CD68抗原。此外,PV-90细胞与患者的原始细胞一样,组成性表达c-fms原癌基因mRNA。患者骨髓细胞中发现三体11(+11),而PV-90细胞最初核型正常,但在4个月时出现了两种不同且独立的染色体异常:90, XX, -Y, -Y, t(9;16) (q11;p13)和90, XX, -Y, -Y, t(17;18) (p13;q21),后者可能涉及p53(17,p13)和bcl-2(18, q21)原癌基因。这些染色体畸变的早期出现与PV-90细胞的遗传不稳定性一致。双谱系标志物的表达和遗传不稳定性可能表明PV-90细胞起源于能够进行髓系和B细胞分化的骨髓增生异常祖细胞的转化。PV-90细胞系可能在许多研究中有用,包括c-fms在细胞分化中的可能作用、人类白血病前期的发病机制以及急性白血病中的谱系混杂。

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