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通过单克隆抗体7.1检测到的大鼠硫酸软骨素蛋白聚糖NG2的人类同源物,可识别伴有t(4;11)(q21;q23)或t(11;19)(q23;p13)以及MLL基因重排的儿童急性淋巴细胞白血病。

Human homologue of the rat chondroitin sulfate proteoglycan, NG2, detected by monoclonal antibody 7.1, identifies childhood acute lymphoblastic leukemias with t(4;11)(q21;q23) or t(11;19)(q23;p13) and MLL gene rearrangements.

作者信息

Behm F G, Smith F O, Raimondi S C, Pui C H, Bernstein I D

机构信息

Department of Pathology, St Jude Children's Research Hospital, Memphis, TN 38105-2794, USA.

出版信息

Blood. 1996 Feb 1;87(3):1134-9.

PMID:8562939
Abstract

Monoclonal antibody 7.1, which recognizes the chondroitin sulfate proteoglycan molecule NG2, was used to screen prospectively blast cells from 104 consecutive children at initial presentation with acute lymphoblastic leukemia (ALL). Reactivity with this antibody was found in 9 cases (8.6%), of whom 5 had a t(4;11)(q21;q23) and 4 had a t(11;19)(p13;q23). None of the NG2- cases had either translocation. Southern blot analysis disclosed MLL gene rearrangement in only the 9 cases with 7.1 reactivity plus the t(4;11)(q21;q23) or t(11;19)(q23;p13) translocation. MLL gene rearrangements were not detected in 89 patient leukemic samples that did not express NG2, including 7 patients with del(11)(q23) or inv(11)(p13q23). As expected from the association with t(4;11) and t(11;19), NG2+ cases were significantly more likely to be infants, to have hyperleukocytosis and central nervous system involvement, to be CD10-, and to express myeloid-associated antigens CD15 and CD65. Despite short follow-up duration, 3 of the NG2+ cases have relapsed while the other 101 patients remain in remission. Thus, blast cell surface expression of NG2 is useful for identifying patients with ALL having t(4;11) or t(11;19) translocations that are associated with poor prognosis, especially in the infant age group.

摘要

单克隆抗体7.1可识别硫酸软骨素蛋白聚糖分子NG2,用于对104例初诊急性淋巴细胞白血病(ALL)儿童的原始细胞进行前瞻性筛查。在9例(8.6%)中发现该抗体呈阳性反应,其中5例有t(4;11)(q21;q23),4例有t(11;19)(p13;q23)。所有NG2阴性病例均无上述易位。Southern印迹分析显示,仅9例7.1反应阳性且伴有t(4;11)(q21;q23)或t(11;19)(q23;p13)易位的病例存在MLL基因重排。在89例不表达NG2的患者白血病样本中未检测到MLL基因重排,包括7例有del(11)(q23)或inv(11)(p13q23)的患者。正如与t(4;11)和t(11;19)相关联所预期的那样,NG2阳性病例更有可能是婴儿,有白细胞增多症和中枢神经系统受累,CD10阴性,并表达髓系相关抗原CD15和CD65。尽管随访时间短,但3例NG2阳性病例已复发,而其他101例患者仍处于缓解期。因此,原始细胞表面表达NG2有助于识别伴有t(4;11)或t(11;19)易位的ALL患者,这些易位与预后不良相关,尤其是在婴儿年龄组。

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