Estey E, Trujillo J M, Cork A, O'Brien S, Beran M, Kantarjian H, Keating M, Freireich E J, Stass S
Department of Hematology, University of Texas M.D. Anderson Cancer Center, Houston 77030.
Hematol Pathol. 1992;6(1):43-8.
Evidence suggests that prognosis in patients with myelodysplastic syndromes (MDS) or acute myelogenous leukemia (AML) depends more on karyotype than on formal classification as either MDS or AML according to the French-American-British (FAB) system. We provide further evidence of overlap between these two entities, reporting 4 patients who presented with either inv(16) (p13q22), del(16) (q22), or t(8;21) despite an FAB diagnosis of MDS rather than the diagnosis of AML with which these abnormalities are generally associated. In 3 patients, disease was relatively long-standing (3-10 months) prior to diagnosis, suggesting that the association between MDS and these cytogenetic abnormalities may not merely reflect a transient phenomenon. Two patients with inv(16) and the MDS subtype refractory anemia with excess blasts in transformation (RAEB-t) received AML-type chemotherapy as did a patient with t(8;21) and RAEB-t. All entered CR paralleling the high CR rate seen in patients with AML and these abnormalities. Our data support the concept that MDS and AML may be different manifestations of the same disease.
有证据表明，骨髓增生异常综合征（MDS）或急性髓性白血病（AML）患者的预后更多地取决于核型，而非按照法美英（FAB）系统进行的MDS或AML的正式分类。我们提供了这两种疾病实体之间存在重叠的进一步证据，报告了4例患者，尽管根据FAB诊断为MDS，但却出现了inv(16)(p13q22)、del(16)(q22)或t(8;21)，而这些异常通常与AML的诊断相关。在3例患者中，疾病在诊断前相对病程较长（3 - 10个月），这表明MDS与这些细胞遗传学异常之间的关联可能不仅仅反映一种短暂现象。2例伴有inv(16)且MDS亚型为转化型原始细胞过多的难治性贫血（RAEB - t）的患者以及1例伴有t(8;21)和RAEB - t的患者接受了AML型化疗。所有患者均进入完全缓解期，这与伴有这些异常的AML患者中所见的高完全缓解率相似。我们的数据支持MDS和AML可能是同一种疾病的不同表现形式这一概念。
