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[费城染色体阳性白血病的细胞遗传学和临床特征]

[Cytogenetic and clinical features of Philadelphia chromosome positive leukemias].

作者信息

Becher R, Kloke O

机构信息

Innere Universitäts- und Poliklinik (Tumorforschung) Westdeutsches Tumorzentrum, Essen.

出版信息

Verh Dtsch Ges Pathol. 1990;74:36-42.

PMID:1708614
Abstract

The Philadelphia chromosome defines chronic myeloid leukemia, and is mostly based on a translocation t(9;22) with a typical BCR-ABL rearrangement which also occurs in so called atypical translocations. The transformation of chronic myeloid leukemia is associated with clonal evolution in 80% of cases. The appearance of an isochromosome 17q unequivocally heralds the onset of a myeloid type of blast crisis. Treatment of Ph-positive CML has still to be considered palliative except for allogeneic bone marrow transplantation. The Philadelphia chromosome is also found in about 20% of patients with acute lymphoblastic leukemia and in about 2% of patients with nonlymphoblastic leukemia. It is associated with a poor prognosis. Molecular and cytogenetic findings help differentiating between de novo acute leukemia and blast crisis of chronic myeloid leukemia.

摘要

费城染色体可确诊慢性髓性白血病,主要基于t(9;22)易位及典型的BCR-ABL重排,这种重排也见于所谓的非典型易位。80%的慢性髓性白血病转化与克隆进化有关。17号等臂染色体的出现明确预示着髓系原始细胞危象的发生。除异基因骨髓移植外,Ph阳性慢性髓性白血病的治疗仍被视为姑息性治疗。约20%的急性淋巴细胞白血病患者和约2%的非淋巴细胞白血病患者也可发现费城染色体。其与预后不良相关。分子和细胞遗传学检查结果有助于区分原发性急性白血病和慢性髓性白血病的原始细胞危象。

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