Philadelphia-chromosome-positive T-lymphoblastic leukemia: Acute leukemia or chronic myelogenous leukemia blastic crisis
Philadelphia-chromosome-positive T-lymphoblastic leukemia: Acute leukemia or chronic myelogenous leukemia blastic crisis
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DOI：
10.1159/000084448
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发表时间：
2005-01-01
影响因子：
2.4
通讯作者：
Ben-Bassat, I
中科院分区：
文献类型：
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作者：
Raanani, P;Trakhtenbrot, L;Ben-Bassat, I
PH1染色体在T细胞性急性淋巴细胞白血病(T-ALL)中的报道很少，这种易位在T-ALL中的临床相关性目前尚不清楚。在慢性粒细胞白血病(CML)中，一些数据表明T细胞来源于白血病克隆，仅有少数T细胞起源的原始细胞危象被报道并经常引起争议。特别是在最初确诊为急变期的病例中，可能很难将这些病例与PH1阳性的T-ALL区分开来。我们在此报告两位患者，他们的临床表现为PH1阳性的T-ALL，并提出了与CML的T细胞急变的鉴别诊断。我们回顾文献并提出有助于诊断的临床和实验室特征。根据我们的文献，报道了23例PH1阳性的T-ALL和44例CML的T细胞急变。这两个实体之间的一些主要差异可能有助于建立CML与PH1阳性T-ALL的PH1阳性T细胞急变期的诊断：男性和年轻在T-ALL中占主导地位。虽然在大多数CML急变期病例中有CML病史，但在T-ALL病例中没有这种病史。T-ALL的骨髓受累均为淋巴母细胞性白血病，但仅有约一半的CML急变期病例。逆转录聚合酶链式反应(RT-PCR)检测的CML急变组中无一例有轻微断裂点转录本，2例T-ALL有轻微断裂点转录本，1例同时有两个转录本。结合形态学和FISH分析可以帮助区分这两个实体，并在我们的一个病例中应用。虽然这两种疾病都有严重的预后，但区分它们可能具有临床意义，特别是在伊马替尼时代。版权所有(C)2005 S.Karger AG，巴塞尔。
The Ph1 chromosome has rarely been reported in T-lineage acute lymphoblastic leukemia (T-ALL), and the clinical relevance of this translocation in T-ALL is currently unknown. In chronic myelogenous leukemia (CML) some data indicate derivation of T-cells from the leukemic clone and only a few cases of T-derived blastic crisis have been reported and quite often disputed. Particularly in cases identified initially in blastic crisis it may be difficult to distinguish those from Ph1-positive T-ALL. We herein report 2 patients who presented with a clinical picture of Ph1-positive T-ALL and who raised a differential diagnosis from T-cell blastic crisis of CML. We review the literature and suggest clinical and laboratory features that can help in the diagnosis. According to our literature review, 23 cases of Ph1-positive T-ALL and 44 cases of T-cell blastic crisis of CML, including ours, were reported. Some major differences between the two entities could help in establishing a diagnosis of Ph1-positive T-cell blastic crisis of CML vs. Ph1-positive T-ALL: Male sex and younger age was more predominant in T-ALL. While in most cases of CML blastic crisis there was a history of CML there was no such history in the T-ALL cases. Medullary involvement with lymphoblastic leukemia was present in all cases of T-ALL but only in about half of the cases of CML blastic crisis. None of the CML-blastic crisis cases tested by RT-PCR showed the minor breakpoint transcript, while 2 cases with T-ALL had the minor breakpoint transcript and 1 had both transcripts. Combined morphologic and FISH analysis can help to distinguish between the two entities and was applied in one of our cases. Although both entities carry a severe prognosis, differentiating between them might have clinical relevance, especially in the imatinib era. Copyright (C) 2005 S. Karger AG, Basel.