Pugh W C, Pearson M, Vardiman J W, Rowley J D
Br J Haematol. 1985 Jul;60(3):457-67. doi: 10.1111/j.1365-2141.1985.tb07443.x.
Morphological re-examination of 25 cases classified as Ph1-negative chronic myelogenous leukaemia (CML), with particular attention to the recent French-American-British (FAB) group proposals for the diagnosis of the myelodysplastic syndromes, led to reclassification of all but one of these cases. Upon review of pretreatment material, seven of the 25 cases (28%) were considered to represent myeloproliferative and reactive conditions other than CML. Of the remaining 18 cases, 17 (94%) were reclassified as myelodysplastic syndromes (MDS). These included one case of refractory anaemia (RA), three of refractory anaemia with excess of blasts (RAEB), four of RAEB in transformation (RAEBT), and nine of chronic myelomonocytic leukaemia (CMMoL). Haematological findings in these 17 patients were compared to those in 50 randomly selected patients with Ph1-positive CML. Whereas an absolute basophilia in the peripheral blood was a nearly constant feature in the Ph1-positive group, it was lacking in all but one of the patients with myelodysplastic syndromes. Differences in the pattern and degree of dysplasia were also noted. Only one of the 25 cases studied was considered morphologically and clinically indistinguishable from Ph1-positive CML. It appears that Ph1-negative CML constitutes a heterogeneous group of prognostically distinct disorders and that, in most instances, careful morphologic examination will permit precise classification.
对25例分类为Ph1阴性慢性粒细胞白血病(CML)的病例进行形态学复查,特别关注法国-美国-英国(FAB)小组最近提出的骨髓增生异常综合征的诊断建议,结果导致除1例之外的所有这些病例都被重新分类。复查预处理材料时,25例中有7例(28%)被认为代表CML以外的骨髓增殖性和反应性疾病。在其余18例中,17例(94%)被重新分类为骨髓增生异常综合征(MDS)。其中包括1例难治性贫血(RA)、3例难治性贫血伴原始细胞增多(RAEB)、4例转化中的RAEB(RAEBT)和9例慢性粒-单核细胞白血病(CMMoL)。将这17例患者的血液学检查结果与50例随机选择的Ph1阳性CML患者的结果进行比较。外周血绝对嗜碱性粒细胞增多在Ph1阳性组几乎是一个恒定特征,而在骨髓增生异常综合征患者中,除1例外,其他患者均无此特征。还注意到发育异常的模式和程度存在差异。所研究的25例中只有1例在形态学和临床上与Ph1阳性CML难以区分。看来Ph1阴性CML构成了一组预后不同的异质性疾病,而且在大多数情况下,仔细的形态学检查将有助于进行精确分类。