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具有正常细胞遗传学的原发性急性髓系白血病是否涉及以红系和/或巨核系发育异常的存在为特征区分的两个主要预后类别?

Do de novo acute myeloid leukemias with normal cytogenetics involve two main prognostic categories distinguished by the presence of erythroblastic and/or megakaryocytic dysplasia?

作者信息

Lemez P, Gáliková J, Haas T

机构信息

Department of Hematology and Blood Transfusion, Hospital Jihlava, Czech Republic.

出版信息

Neoplasma. 2000;47(1):41-7.

Abstract

De novo acute myeloid leukemias (AML) patients with normal cytogenetics represent a standard risk cytogenetic group. Erythroblastic and/or megakaryocytic dysplasia (EMD) in diagnostic bone marrow smears of 28 consecutive AML patients with a normal karyotype was studied. Twelve patients 21-85 (median 48) years old were categorized without EMD, 14 patients 34-90 (median 58) years old with EMD, and 2 patients were not evaluable for EMD. One cycle of induction therapy 4 + 7, with 4 doses of daunorubicin 45 mg/m2/d and standard doses of cytosine arabinoside for 7 days induced 10 complete and 2 partial remissions in 12 cases without EMD but lead to only one complete remission, 6 non-responses and 3 induction deaths in 10 cases with EMD (p = 0.002). However, high doses of cytosine arabinoside plus daunorubicin induced complete remission in 6 of 7 patients with EMD. In patients under 66 years treated by intensive consolidations the estimate of median survival was 50.6 months in 10 cases without EMD, significantly higher than 8.0 months in 11 cases with EMD (p = 0.043). De novo AML with normal cytogenetics might be divided into two biological categories, the first favorable-risk category without EMD and the second poor-risk category with EMD.

摘要

细胞遗传学正常的初发急性髓系白血病(AML)患者属于标准风险细胞遗传学组。对连续28例核型正常的AML患者诊断性骨髓涂片的红系和/或巨核系发育异常(EMD)进行了研究。12例年龄在21 - 85岁(中位年龄48岁)的患者被归类为无EMD,14例年龄在34 - 90岁(中位年龄58岁)的患者有EMD,2例患者无法评估EMD。采用4 + 7诱导治疗方案,即4剂柔红霉素45 mg/m²/d和标准剂量阿糖胞苷治疗7天,在12例无EMD的患者中诱导出10例完全缓解和2例部分缓解,但在10例有EMD的患者中仅诱导出1例完全缓解、6例未缓解和3例诱导死亡(p = 0.002)。然而,高剂量阿糖胞苷加柔红霉素在7例有EMD的患者中使6例获得完全缓解。在接受强化巩固治疗的66岁以下患者中,10例无EMD患者的中位生存估计为50.6个月,显著高于11例有EMD患者的8.0个月(p = 0.043)。细胞遗传学正常的初发AML可能分为两个生物学类别,第一类是无EMD的低风险类别,第二类是有EMD的高风险类别。

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