Germing U, Gattermann N, Minning H, Heyll A, Aul C
Department of Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University, Düsseldorf, Germany.
Leuk Res. 1998 Oct;22(10):871-8. doi: 10.1016/s0145-2126(97)00192-6.
The FAB group proposed to distinguish two subgroups of chronic myelomonocytic leukemia (CMML). Depending on the total leukocyte count, a myelodysplastic type (MDS-CMML) (< or = 13,000 microl(-1)) was separated from a myeloproliferative type (MPD-CMML) (> 13,000 microl(-1)). Based on retrospective analyses of 158 patients with CMML, we compared the presenting clinical and hematological features of both disorders and examined whether the refined classification is important in terms of prognosis. There were 81 patients with MDS-CMML and 77 patients with MPD-CMML. Median age of patients at diagnosis (70 versus 72 years) was not different. The sex ratio showed a preponderance of males in the MPD group (m:f; 2.1:1). Splenomegaly was more common in MPD-CMML (54 versus 30%; P = 0.002). With regard to laboratory findings, patients with MPD-CMML presented with significantly higher LDH values (medians 295 versus 231 U ml(-1); P = 0.008) and higher serum deoxythymidine kinase levels (medians 150 versus 41 U microl(-1); P = 0.0025). Except for white blood cell count (WBC), peripheral blood counts were not different. Median percentage of bone marrow blasts was 9% and cumulative survival rates were similar in both disorders. Two years after diagnosis, actuarial survival for patients with MPD-CMML was 33%, as compared to 50% for patients with MDS-CMML (P = 0.31). The probability of transformation to AML was higher in MDS-CMML (32 versus 17% after 5 years), but this difference also did not reach statistical significance. The survival of patients with MDS-CMML was similar to that of other MDS patients (RAEB) who had corresponding medullary blast counts. Using the Düsseldorf-score, we could define two risk groups within MDS-CMML with a median survial of 12 versus 40 months (P = 0.001). None of the known scoring systems could define risk groups within the MPD-CMML group. In summary, these data suggest that MDS-CMML and MPD-CMML are clinically distinguishing conditions, but the separation provides little prognostic information. Further studies are needed to clarify whether response to therapy is different in MDS-CMML and MPD-CMML.
FAB小组提议区分慢性粒单核细胞白血病（CMML）的两个亚组。根据白细胞总数，将骨髓增生异常型（MDS-CMML）（≤13,000/μl）与骨髓增殖型（MPD-CMML）（>13,000/μl）区分开来。基于对158例CMML患者的回顾性分析，我们比较了这两种疾病的临床和血液学特征，并研究了这种精细分类在预后方面是否重要。其中有81例MDS-CMML患者和77例MPD-CMML患者。诊断时患者的中位年龄（70岁对72岁）无差异。性别比显示MPD组男性占优势（男：女；2.1：1）。脾肿大在MPD-CMML中更常见（54%对30%；P = 0.002）。关于实验室检查结果，MPD-CMML患者的乳酸脱氢酶（LDH）值显著更高（中位数295对231 U/ml；P = 0.008），血清脱氧胸苷激酶水平也更高（中位数150对41 U/μl；P = 0.0025）。除白细胞计数（WBC）外，外周血细胞计数无差异。骨髓原始细胞的中位百分比为9%，两种疾病的累积生存率相似。诊断后两年，MPD-CMML患者的精算生存率为33%，而MDS-CMML患者为50%（P = 0.31）。MDS-CMML转化为急性髓系白血病（AML）的概率更高（5年后为32%对17%），但这种差异也未达到统计学显著性。MDS-CMML患者的生存率与其他具有相应骨髓原始细胞计数的骨髓增生异常综合征（MDS）患者（难治性贫血伴原始细胞增多症，RAEB）相似。使用杜塞尔多夫评分，我们可以在MDS-CMML中定义两个风险组，中位生存期分别为12个月和40个月（P = 0.001）。没有已知的评分系统能够在MPD-CMML组中定义风险组。总之，这些数据表明MDS-CMML和MPD-CMML在临床上是有区别的情况，但这种区分提供的预后信息很少。需要进一步研究以阐明MDS-CMML和MPD-CMML对治疗的反应是否不同。
