Hanamura A, Ichikawa A
Department of Haematology, Gifu Prefectural Tajimi Hospital.
Rinsho Ketsueki. 1995 Aug;36(8):755-61.
A 64-year-old woman was diagnosed as having myelodysplastic syndrome (MDS) at 45 months after receiving radiotherapy for advanced carcinoma of the uterine cervix. We chose low dose therapy of SPAC and ACR because of the diagnosis as therapy-related MDS and the existence of radiation colitis. She obtained minor response, but two months later she transformed to AML (M2). The interval between low dose therapies was getting shorter and shorter, so we tried intensive chemotherapy consisting of BHAC, ACR and 6MP. Blast numbers were reduced, but she died of sepsis and intestinal bleeding. The patients of MDS with t(8;21) and the patients of therapy-related AML (tAML) with t(8;21) are very rare. According to the literature, only karyotype is a prognostic factor in AML/MDS with t(8;21). And diagnosis by the criteria of FAB classification is of little value regarding clinical progress. That is to say, if the patient has only t(8;21) or karyotypic abnormalities which are of little value in prognosis, such as the loss of a sex chromosome, it must be treated as de novo AML, but if patient has karyotypic abnormalities such as -5, 5q-, -7, 7q-, and/or multiple (complicated) abnormalities, we must accept that the prognosis is poor and must treat it as ordinary MDS/tAML.
一名64岁女性在接受晚期宫颈癌放射治疗45个月后被诊断为骨髓增生异常综合征（MDS）。由于诊断为治疗相关的MDS且存在放射性结肠炎，我们选择了小剂量的SPAC和ACR治疗。她获得了轻微缓解，但两个月后转化为急性髓系白血病（AML，M2型）。小剂量治疗之间的间隔越来越短，因此我们尝试了由BHAC、ACR和6-巯基嘌呤组成的强化化疗。原始细胞数量减少了，但她死于败血症和肠道出血。伴有t(8;21)的MDS患者以及伴有t(8;21)的治疗相关急性髓系白血病（tAML）患者非常罕见。根据文献，在伴有t(8;21)的AML/MDS中，只有核型是一个预后因素。而按照FAB分类标准进行诊断对临床进展的价值不大。也就是说，如果患者仅存在t(8;21)或对预后价值不大的核型异常，如性染色体丢失，必须将其视为原发性AML，但如果患者存在-5、5q-、-7、7q-和/或多种（复杂）异常等核型异常，我们必须承认预后较差，必须将其视为普通的MDS/tAML进行治疗。
