Urbano-Ispizua A, Cervantes F, Matutes E, Villamor N, Pujades A, Sierra J, Feliu E, Vives-Corrons J L, Montserrat E, Rozman C
Postgraduate School of Haematology, Farreras Valentí, Hospital Clínic de Barcelona, Spain.
Leukemia. 1993 Sep;7(9):1349-54.
Blast cells from 40 patients with Philadelphia-positive chronic myeloid leukaemia (CML) in blast crisis were analysed by immunophenotypic methods. In 27 cases, BCR gene studies were also performed. By light microscopy morphology and cytochemistry the cases were classified as follows: undifferentiated (n = 7; 17.5%), myeloid (n = 27; 67.5%), and lymphoid (n = 6; 15%). On the basis of the immunological markers, the cases were reclassified as: myeloid (n = 17; 42.5%), megakaryoblastic (n = 17; 42.5%), and lymphoid (n = 6; 15%). The seven cases initially considered as undifferentiated by morphological and conventional cytochemical criteria were classified as myeloid (four cases) and megakaryoblastic (three cases) by marker analysis. The monoclonal antibody anti-myeloperoxidase (anti-MPO) was the most sensitive myeloid associated marker in these cases, being positive in five of them. A significant proportion (27%) of non-lymphoid blast crisis cases were CD7-positive, and myeloid markers were positive in the four lymphoid CML-CB cases studied. Analysis of the clinico-haematological characteristics on the various subgroups of patients showed that patients with lymphoid blast crisis had shorter duration of the chronic phase, more frequent extramedullary blastic involvement, more favourable response to therapy, and longer survival. Finally, a trend for an association between megakaryoblastic involvement of blast crisis and breakpoint localization in the 3' extreme of the M-bcr segment was also noted.
采用免疫表型分析方法对40例处于急变期的费城染色体阳性慢性髓性白血病(CML)患者的原始细胞进行了分析。其中27例还进行了BCR基因研究。通过光学显微镜形态学和细胞化学方法,将这些病例分类如下:未分化型(n = 7;17.5%)、髓系型(n = 27;67.5%)和淋巴系型(n = 6;15%)。根据免疫标记物,这些病例重新分类为:髓系型(n = 17;42.5%)、巨核母细胞型(n = 17;42.5%)和淋巴系型(n = 6;15%)。最初根据形态学和传统细胞化学标准被认为是未分化型的7例病例,通过标记分析分类为髓系型(4例)和巨核母细胞型(3例)。单克隆抗体抗髓过氧化物酶(抗MPO)是这些病例中最敏感的髓系相关标记物,其中5例呈阳性。相当比例(27%)的非淋巴系急变期病例CD7呈阳性,在研究的4例淋巴系CML-CB病例中髓系标记物呈阳性。对患者各亚组临床血液学特征的分析表明,淋巴系急变期患者的慢性期持续时间较短,髓外原始细胞浸润更频繁,对治疗的反应更良好,生存期更长。最后,还注意到急变期巨核母细胞受累与M-bcr区段3'末端断点定位之间存在关联的趋势。