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成人急性白血病患者中的费城染色体(Ph1):Ph1阳性与Ph1阴性患者的比较

The Philadelphia chromosome (Ph1) in adults presenting with acute leukaemia: a comparison of Ph1+ and Ph1-patients.

作者信息

Bloomfield C D, Peterson L C, Yunis J J, Brunning R D

出版信息

Br J Haematol. 1977 Jul;36(3):347-58. doi: 10.1111/j.1365-2141.1977.tb00658.x.

Abstract

To evaluate the frequency and clinical significance of the Philadelphia chromosome (Ph1) in adult acute leukaemia, bone marrow chromosomes were studied in 15 adults with acute lymphocytic leukaemia (ALL) and 55 with acute nonlymphocytic leukaemia (ANLL). Morphology, clinical findings, therapeutic response and survival were compared in patients with and without the Ph1. The Ph1 was found in six newly diagnosed adults presenting with ALL. Adults with Ph1+ ALL differed from those with Ph1-ALL in being older, in having more frequent lymphadenopathy and splenomegaly and in demonstrating higher initial leucocyte counts and more peripheral blasts. Complete remissions were obtained in all nine adults with Ph1-All but in only three of six with Ph1+ ALL. Adults with Ph1-ALL survived significantly longer. Four adults with ANALL were Ph1+. They did not respond to treatment and survived significantly shorter periods than adults with Ph1-ANLL. No clinical or morphologic features indicated which patients with acute leukaemia would have the Ph1. Since the presence of the Ph1 in acute leukaemia has therapeutic and prognostic significance, marrow chromosome studies should be performed in adults presenting with acute leukaemia, especially ALL.

摘要

为评估费城染色体(Ph1)在成人急性白血病中的出现频率及临床意义,我们对15例成人急性淋巴细胞白血病(ALL)患者和55例成人急性非淋巴细胞白血病(ANLL)患者的骨髓染色体进行了研究。比较了有或无Ph1的患者的形态学、临床发现、治疗反应及生存率。在6例新诊断的成人ALL患者中发现了Ph1。Ph1阳性的成人ALL患者与Ph1阴性的患者不同,前者年龄较大,淋巴结病和脾肿大更为常见,初始白细胞计数更高,外周血原始细胞更多。9例Ph1阴性的成人ALL患者均获得完全缓解,但6例Ph1阳性的患者中只有3例获得完全缓解。Ph1阴性的成人ALL患者生存时间显著更长。4例ANLL患者为Ph1阳性。他们对治疗无反应,生存时间比Ph1阴性的ANLL患者显著更短。没有临床或形态学特征可提示哪些急性白血病患者会有Ph1。由于急性白血病中Ph1的存在具有治疗和预后意义,因此对于成人急性白血病患者,尤其是ALL患者,应进行骨髓染色体研究。

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