Béné M C, Bernier M, Casasnovas R O, Castoldi G, Doekharan D, van der Holt B, Knapp W, Lemez P, Ludwig W D, Matutes E, Orfao A, Schoch C, Sperling C, van't Veer M B
GEIL-laboratoire d'Immunologie, Faculté de Medicine, Nancy, France.
Br J Haematol. 2001 Jun;113(3):737-45. doi: 10.1046/j.1365-2141.2001.02801.x.
Haematological, immunophenotypic and cytogenetic characteristics were analysed in 241 patients with acute myeloid leukaemia (AML) M0, including 58 children. Children < 3 years and adults between 60 and 70 years of age were most frequently affected. Immunophenotyping showed a heterogeneous phenotype. Anti-myeloperoxidase was positive in about half of the patients. Cytogenetic data were available from 129 (54%) patients. A normal karyotype was found in only 24%. Most of the abnormalities were unbalanced and the chromosomes 5, 7, 8 and 11 were the most frequently affected. Survival data were available from 152 treated patients (63%). The median overall survival for all patients was 10 months, 20 months for children (n = 36), 10 months for the young adult group (n = 50) and 7 months for the elderly patients (n = 66) (P = 0.09). Karyotype was not a prognostic factor influencing survival. AML M0 shows the immunological characteristics of early progenitor cells, but the expression of the different markers and cytogenetic abnormalities is heterogeneous. The prognosis is poor compared with other de novo AML and similar to that of AML with multilineage dysplasia or AML following myelodysplastic syndromes.
对241例急性髓系白血病(AML)M0患者进行了血液学、免疫表型和细胞遗传学特征分析,其中包括58例儿童患者。3岁以下儿童和60至70岁的成年人受影响最为频繁。免疫表型分析显示出异质性表型。约一半患者抗髓过氧化物酶呈阳性。129例(54%)患者有细胞遗传学数据。仅24%患者核型正常。大多数异常为不平衡型,5号、7号、8号和11号染色体受影响最为频繁。152例接受治疗的患者(63%)有生存数据。所有患者的中位总生存期为10个月,儿童(n = 36)为20个月,年轻成人组(n = 50)为10个月,老年患者(n = 66)为7个月(P = 0.09)。核型不是影响生存的预后因素。AML M0显示早期祖细胞的免疫特征,但不同标志物的表达和细胞遗传学异常是异质性的。与其他初发AML相比,其预后较差,与伴有多系发育异常的AML或骨髓增生异常综合征后的AML相似。