Pedersen-Bjergaard J, Philip P, Pedersen N T, Hou-Jensen K, Svejgaard A, Jensen G, Nissen N I
Cancer. 1984 Aug 1;54(3):452-62. doi: 10.1002/1097-0142(19840801)54:3<452::aid-cncr2820540313>3.0.co;2-9.
Secondary acute nonlymphocytic leukemia or its earlier stages, preleukemia or an acute myeloproliferative syndrome with refractory cytopenia and clonal cytogenetic abnormalities of the bone marrow, was diagnosed in 55 patients previously treated for other malignant diseases. In patients with overt leukemia, cytologic, and cytochemical studies showed predominance of the French-American-British (FAB) type M2. Cytogenetic examination demonstrated a normal karyotype in 11 cases, whereas clonal abnormalities were observed in 44 patients. Defects of chromosome 7 were observed in 24 cases, most often -7, and defects of chromosome 5 in 14 cases, most often 5q-. In addition, chromosomes 3 and 17 were possibly nonrandomly involved. Other abnormalities commonly observed in de novo acute nonlymphocytic leukemia as t(8;21) and t(15;17) were not observed and +8 rarely seen in secondary leukemia. The survival from the leukemic complication was short for the whole group of 55 patients (median, 7 months). However, a significantly longer survival was observed in a subgroup of 11 patients with a normal karyotype (P less than 0.01), due to a favorable response to antileukemic chemotherapy, and in a subgroup of 11 patients with -7 or -C as the only cytogenetic abnormality (P less than 0.01), due to a prolonged preleukemic phase, compared with the remaining 33 cases with mostly multiple karyotypic abnormalities. Three preleukemic patients with -7 who were studied during transformation to overt leukemia all developed additional cytogenetic abnormalities. According to the two-step or multistep hypothesis for malignant transformation, the prolonged preleukemic course in patients with -7 as the only abnormality could represent a premalignant stage, in which further evolution is required for development of overt leukemia. The patients showed a random distribution of blood groups and HLA types.
55例曾接受其他恶性疾病治疗的患者被诊断为继发性急性非淋巴细胞白血病或其早期阶段，即白血病前期或伴有难治性血细胞减少和骨髓克隆性细胞遗传学异常的急性骨髓增殖综合征。在明显白血病患者中，细胞学和细胞化学研究显示法美英（FAB）M2型占优势。细胞遗传学检查显示11例核型正常，而44例患者观察到克隆性异常。24例观察到7号染色体缺陷，最常见的是-7，14例观察到5号染色体缺陷，最常见的是5q-。此外，3号和17号染色体可能非随机受累。未观察到初发性急性非淋巴细胞白血病中常见的其他异常，如t(8;21)和t(15;17)，继发性白血病中+8也很少见。55例患者总体因白血病并发症导致的生存期较短（中位数为7个月）。然而，11例核型正常的亚组患者生存期明显更长（P<0.01），这归因于对抗白血病化疗的良好反应；11例以-7或-C作为唯一细胞遗传学异常的亚组患者生存期也明显更长（P<0.01），这归因于白血病前期阶段延长，与之相比，其余33例大多有多种核型异常的患者生存期较短。3例白血病前期伴有-7的患者在转化为明显白血病期间进行研究，均出现了额外的细胞遗传学异常。根据恶性转化的两步或多步假说，以-7作为唯一异常的患者白血病前期病程延长可能代表一个癌前阶段，在此阶段需要进一步演变才能发展为明显白血病。患者血型和HLA类型呈随机分布。
