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[Ph1 阳性白血病:细胞遗传学概述与预后]

[Ph1-positive leukemia: cytogenetic outline and prognosis].

作者信息

Misawa S

机构信息

Third Department of Medicine, Kyoto Prefectural University of Medicine.

出版信息

Nihon Rinsho. 1992 Jun;50(6):1274-80.

PMID:1518145
Abstract

Ph1-positive leukemias consist of acute leukemia (Ph1 AL) and CML. Cytogenetically, Ph1 AL is often associated with +6, -7, +8, +21, or +Ph1. CML is predominantly accompanied by +Ph1, +8, i (17q), +19 in myeloid crisis and +Ph1, +8, +21 in lymphoid crisis. Thus, i(17q) seems specific for myeloid crisis of CML. Ph1 constricts ABL/BCR within M-BCR in CML and in one half of the adult Ph1 AL. BCR breaks upstream to M-BCR in the other half of adult AL and in most of childhood AL. However, the breakpoint does not affect clinical and hematological features in AL. Consequently, there seems to be two types of Ph1 leukemia; one is AL representing m-BCR rearrangement and the other is CML and Ph1 AL showing M-BCR rearrangement.

摘要

Ph1 阳性白血病包括急性白血病(Ph1 急性白血病)和慢性粒细胞白血病(CML)。细胞遗传学上,Ph1 急性白血病常与 +6、-7、+8、+21 或 +Ph1 相关。CML 在髓系危象时主要伴有 +Ph1、+8、i(17q)、+19,在淋巴系危象时伴有 +Ph1、+8、+21。因此,i(17q) 似乎是 CML 髓系危象的特异性表现。在 CML 以及一半的成人 Ph1 急性白血病中,Ph1 在 M-BCR 内使 ABL/BCR 融合。在另一半成人急性白血病和大多数儿童急性白血病中,BCR 在 M-BCR 上游断裂。然而,该断裂点并不影响急性白血病的临床和血液学特征。因此,似乎存在两种类型的 Ph1 白血病;一种是代表 m-BCR 重排的急性白血病,另一种是 CML 和 Ph1 急性白血病,表现为 M-BCR 重排。

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