Criel A, Verhoef G, Vlietinck R, Mecucci C, Billiet J, Michaux L, Meeus P, Louwagie A, Van Orshoven A, Van Hoof A, Boogaerts M, Van den Berghe H, De Wolf-Peeters C
Department of Haematology, A.Z. St Jan Brugge, Belgium.
Br J Haematol. 1997 May;97(2):383-91. doi: 10.1046/j.1365-2141.1997.402686.x.
We analysed a group of 390 patients, diagnosed with chronic lymphocytic leukaemia (CLL). Cases were subclassified as morphologically typical and atypical CLL according to the criteria of the FAB proposal. Typical CLL cases were mostly diagnosed at a low-risk stage (Binet A/Rai 0), required no immediate treatment and expected a long survival; atypical CLL cases mostly presented at a more advanced risk stage (Binet B/Rai I-II), usually required immediate treatment and their survival was shorter. Moreover, clinical staging was of prognostic significance in typical but not in atypical cases. In typical CLL, del(11q) was the most common chromosomal abnormality (21%) whereas in atypical CLL trisomy 12 was found in about 65% of the cases documented with an abnormal karyotype. Although chromosomal abnormalities were associated with a poor survival in typical CLL, they are of no prognostic significance in atypical CLL. Based on these data, we conclude that subtyping CLL by morphology enables the identification of two groups of cases, each characterized by a specific clinical presentation, different cytogenetic abnormalities and prognostic parameters. We speculate that these two groups may represent two related, but different, diseases with different prognostic parameters and a different survival.
我们分析了一组390例被诊断为慢性淋巴细胞白血病（CLL）的患者。根据FAB方案的标准，病例被分为形态学典型和非典型CLL。典型CLL病例大多在低风险阶段（Binet A/Rai 0）被诊断，无需立即治疗，预期生存期长；非典型CLL病例大多在更高风险阶段（Binet B/Rai I-II）出现，通常需要立即治疗，生存期较短。此外，临床分期在典型病例中有预后意义，而在非典型病例中则没有。在典型CLL中，del(11q)是最常见的染色体异常（21%），而在非典型CLL中，约65%有核型异常记录的病例发现有12号染色体三体。虽然染色体异常与典型CLL的不良生存相关，但在非典型CLL中它们没有预后意义。基于这些数据，我们得出结论，通过形态学对CLL进行亚型分类能够识别出两组病例，每组病例具有特定的临床表现、不同的细胞遗传学异常和预后参数。我们推测这两组病例可能代表两种相关但不同的疾病，具有不同的预后参数和生存期。
