Michels S D, Saumur J, Arthur D C, Robison L L, Brunning R D
Departments of Laboratory Medicine, University of Minnesota Health Sciences Center, Minneapolis 55455.
Cancer. 1989 Dec 1;64(11):2340-6. doi: 10.1002/1097-0142(19891201)64:11<2340::aid-cncr2820641125>3.0.co;2-i.
This study consists of 52 patients whose blood and bone marrow findings fulfilled the French-American-British (FAB) Cooperative Group criteria for a diagnosis of refractory anemia with excess of blasts in transformation (RAEB-T). The basis for the diagnosis of RAEB-T included 37 patients (71%) meeting one criterion, 13 patients (25%) meeting two criteria, and two patients (4%) meeting three criteria; 44% met only the criterion of the detection of Auer rods. Clonal chromosome abnormalities were seen in seven of 16 cases studied and included abnormalities of chromosome 5, an extra 8, and complex abnormalities. Thirty-one patients were treated with a chemotherapeutic regimen that included an anthracycline and cytarabine; 13 of 17 patients (77%) 45 years of age or younger and six of 14 patients (43%) over 45 years of age achieved CR. The median survival for the 17 patients 45 years of age or younger is 25+ months; the median survival for the 14 patients over 45 years of age is 12.3 months. The median survival for 13 untreated patients over the age of 45 is 4.5 months. The process in 18 of the 52 patients (35%) evolved to overt acute myeloid leukemia (AML) in 1 to 38 months after diagnosis of RAEB-T, in seven of 20 patients (35%) 45 years of age or younger, and 11 of 32 patients (34%) over 45 years. Fifteen of the 18 patients received antileukemic therapy in the RAEB-T phase. There were no clinical or laboratory features that were reliably predictive of transformation to AML.
本研究包含52例患者，其血液和骨髓检查结果符合法美英（FAB）协作组关于转化型原始细胞过多难治性贫血（RAEB-T）的诊断标准。RAEB-T的诊断依据包括：37例患者（71%）符合一项标准，13例患者（25%）符合两项标准，2例患者（4%）符合三项标准；44%的患者仅符合检测到奥氏小体这一标准。在16例研究病例中，7例出现克隆性染色体异常，包括5号染色体异常、额外的8号染色体以及复杂异常。31例患者接受了包含蒽环类药物和阿糖胞苷的化疗方案；17例45岁及以下患者中有13例（77%）达到完全缓解（CR），14例45岁以上患者中有6例（43%）达到CR。17例45岁及以下患者的中位生存期为25 +个月；14例45岁以上患者的中位生存期为12.3个月。13例45岁以上未接受治疗患者的中位生存期为4.5个月。52例患者中有18例（35%）在RAEB-T诊断后的1至38个月内病情进展为明显的急性髓系白血病（AML），其中20例45岁及以下患者中有7例（35%），32例45岁以上患者中有11例（34%）。18例患者中有15例在RAEB-T阶段接受了抗白血病治疗。没有可靠预测向AML转化的临床或实验室特征。
