Germing Ulrich, Strupp Corinna, Kuendgen Andrea, Aivado Manuel, Giagounidis Aristoteles, Hildebrandt Barbara, Aul Carlo, Haas Rainer, Gattermann Norbert
Department of Haematology, Oncology and Clinical Immunology, Heinrich-Heine-University, Düsseldorf, Germany.
Br J Haematol. 2006 Jan;132(2):162-7. doi: 10.1111/j.1365-2141.2005.05853.x.
The French-American-British (FAB) classification assigns patients with myelodysplastic syndromes to the category of refractory anaemia with excess blasts (RAEB) if they have a medullary blast count of 5-20%, and/or a peripheral blast count of 2-5%. The new World Health Organization (WHO) classification subdivides RAEB into RAEB I with a medullary blast count < or =10% and a peripheral blast count < or =5% and RAEB II with >10% medullary and/or >5% peripheral blasts. RAEB II is also diagnosed if Auer rods are present. In 558 patients, we analysed these subtypes of RAEB in terms of haematological characteristics, karyotype anomalies and prognosis. RAEB I was diagnosed in 256 and RAEB II in 302 patients. In the RAEB II group, 22% of patients had >5% peripheral blasts or the presence of Auer rods. The median survival was 16 months for RAEB I as compared with 9 months for RAEB II. Patients with Auer rods, regardless of their medullary and peripheral blast count, had no worse prognosis. No significant differences were identified between the RAEB subtypes with respect to clinical, morphological, haematological and cytogenetic parameters. The survival data support the WHO reclassification of RAEB based on peripheral and medullary blast counts and Auer rods. The WHO classification is useful for diagnosis and provides risk stratification, supported by cytogenetic data for clinical decision making, identifying those RAEB patients with an unfavourable prognosis who should be offered chemotherapy or stem cell transplantation.
法国-美国-英国(FAB)分类法规定,骨髓增生异常综合征患者若骨髓原始细胞计数为5%-20%,和/或外周血原始细胞计数为2%-5%,则归类为伴有过多原始细胞的难治性贫血(RAEB)。世界卫生组织(WHO)新分类法将RAEB再细分为:RAEB I(骨髓原始细胞计数≤10%且外周血原始细胞计数≤5%)和RAEB II(骨髓原始细胞计数>10%和/或外周血原始细胞计数>5%)。若存在奥氏小体,也诊断为RAEB II。我们分析了558例患者的这些RAEB亚型的血液学特征、核型异常及预后。256例患者诊断为RAEB I,302例患者诊断为RAEB II。在RAEB II组中,22%的患者外周血原始细胞>5%或存在奥氏小体。RAEB I的中位生存期为16个月,而RAEB II为9个月。有奥氏小体的患者,无论其骨髓和外周血原始细胞计数如何,预后都不差。RAEB各亚型在临床、形态学、血液学和细胞遗传学参数方面未发现显著差异。生存数据支持WHO基于外周血和骨髓原始细胞计数及奥氏小体对RAEB进行的重新分类。WHO分类法有助于诊断并提供风险分层,细胞遗传学数据支持其用于临床决策,可识别出预后不良的RAEB患者,这些患者应接受化疗或干细胞移植。