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T细胞急性淋巴细胞白血病及相关的碱性螺旋-环-螺旋基因SCL/tal

T-cell acute lymphoblastic leukemia and the associated basic helix-loop-helix gene SCL/tal.

作者信息

Goldfarb A N, Greenberg J M

机构信息

Case Western Reserve University, Institute of Pathology, Cleveland, Ohio 44106-4943.

出版信息

Leuk Lymphoma. 1994 Jan;12(3-4):157-66. doi: 10.3109/10428199409059586.

Abstract

T-cell acute lymphoblastic leukemia (T-ALL) is a relatively uncommon disease, constituting only approximately 15% of newly diagnosed acute lymphoblastic leukemias (ALL) in the United States, or roughly 300 cases per year. Outside of the United States, in countries such as Egypt and India, T-ALL may represent as much as 50% of all ALL's but still remains an overall rare disease. The clinical importance of T-ALL lies in its poor responsiveness to therapy that has proved highly effective with standard B-cell precursor ALL (BCP-ALL). The scientific importance of human T-ALL has resided in its role as a cancer prototype, permitting the identification of novel genes centrally involved in both neoplastic change and normal cellular differentiation. One of these genes, SCL/tal, has received significant attention due to its intimate involvement in T-ALL, as well as in normal hematopoiesis. Although a tremendous amount has been recently discovered about SCL/tal, its exact roles in leukemogenesis and normal hematopoiesis remain obscure.

摘要

T细胞急性淋巴细胞白血病(T-ALL)是一种相对罕见的疾病,在美国新诊断的急性淋巴细胞白血病(ALL)中仅占约15%,即每年约300例。在美国以外的国家,如埃及和印度,T-ALL在所有ALL中可能占比高达50%,但总体上仍然是一种罕见疾病。T-ALL的临床重要性在于其对治疗的反应较差,而标准的B细胞前体ALL(BCP-ALL)治疗已被证明非常有效。人类T-ALL的科学重要性在于它作为癌症原型的作用,有助于识别在肿瘤发生和正常细胞分化中起核心作用的新基因。其中一个基因SCL/tal因其与T-ALL以及正常造血密切相关而受到了极大关注。尽管最近对SCL/tal有了大量发现,但其在白血病发生和正常造血中的确切作用仍不清楚。

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