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首页> 外文期刊>Pediatric Research >CLINICAL IMMUNOLOGICAL AND CYTOGENETIC FEATURES OF Ph1 POSITIVE CHRONIC GRANULOCYTIC LEUKAEMIA IN CHILDHOOD
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CLINICAL IMMUNOLOGICAL AND CYTOGENETIC FEATURES OF Ph1 POSITIVE CHRONIC GRANULOCYTIC LEUKAEMIA IN CHILDHOOD

机译:儿童Ph1阳性慢性粒细胞性白血病的临床免疫学和细胞遗传学特征

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摘要

Adult (Ph1 positive) chronic granulocytic leukaemia was diagnosed in eight of 123 (6%) consecutive new cases of childhood leukaemia. Four patients presented as typical CGL and four in blast crisis; two of the four chronic cases have since developed blast crisis. Morphological characterization of the blasts in these six acute cases suggested a myeloblastic crisis in one and a lymphoblastic crisis in five. Study of ‘membrane markers’ indicated one myeloid, four lymphoblastic and one mixed lymphoid/myeloid crisis. Remission induction was easily achieved in two of the five lymphoblastic cases; one child relapsed rapidly and the other remains well eighteen months after conversion but serial cytogenetic studies show persistence of the Ph1 chromosome.We conclude that adult CGL in childhood presents frequently as blast crisis and may even mimic ‘good risk’ ALL. Response to treatment cannot be predicted by morphological or immunologies1 characteristics.
机译:在连续的123例儿童白血病新病例中,有8例诊断为成人(Ph1阳性)慢性粒细胞白血病。四例患者表现为典型的CGL,四例发生爆炸危险;此后,四个慢性病例中有两个已经发生爆炸危机。在这6例急性病例中,爆炸的形态学特征表明,其中1例为骨髓幼细胞危机,5例为淋巴母细胞危机。对“膜标记物”的研究表明有1个髓样,4个淋巴细胞和1个混合的淋巴样/髓样危机。在5例淋巴母细胞病例中,有2例可轻松实现缓解。一个孩子迅速复发,另一个孩子在转换后仍然存活了18个月,但是连续的细胞遗传学研究显示Ph1染色体的持久性。我们的结论是,成年CGL在儿童时期经常表现为爆炸危险,甚至可能模仿“好风险” ALL。无法通过形态学或免疫学特征预测对治疗的反应。

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