Lemez P, Michalová K, Zemanová Z, Marinov I, Trpáková A, Moravcová J, Jelínek J
Institute of Hematology and Blood Transfusion, Prague, Czech Republic.
Leuk Res. 1998 Jul;22(7):581-8. doi: 10.1016/s0145-2126(97)00177-x.
We report three cases of acute myeloid leukemia (AML) with a near-tetraploid karyotype in most metaphases while lacking chromosomal abnormalities typical for AML. All patients, 63, 72 and 81 years old, were female. In two cases, AML was diagnosed 5-7 months after a cytopenic period while the third patient had a secondary AML after therapy for a pleural tumor. Leukemic blasts were classified as AML M0, AML M1 and AML without further specification. Two patients died on the 18th and 52nd day after the start of cytotoxic chemotherapy, the third patient refused chemotherapy and died 22 days after the diagnosis. The three patients may represent a distinct AML category with the following features: (1) the near-tetraploid karyotype in most bone marrow metaphases examined at diagnosis of AML; (2) the presence of very large myeloid blasts in the bone marrow and dysplastic changes in erythroid and/or megakaryocytic lineages pointing to the origin of AML in pluripotent myeloid progenitor cells; (3) the expression of the CD34 antigen; (4) the low growth of granulocyte-macrophage colony forming cells in culture; and (5) the presence of a preleukemic phase, a higher age and a poor prognosis.
我们报告了三例急性髓系白血病（AML）患者，其多数中期细胞具有近四倍体核型，同时缺乏AML典型的染色体异常。所有患者均为女性，年龄分别为63岁、72岁和81岁。其中两例患者在血细胞减少期5 - 7个月后被诊断为AML，第三例患者在胸膜肿瘤治疗后发生继发性AML。白血病原始细胞分别被分类为AML M0、AML M1以及未进一步明确分类的AML。两名患者在细胞毒性化疗开始后的第18天和第52天死亡，第三名患者拒绝化疗，在诊断后22天死亡。这三名患者可能代表一种具有以下特征的独特AML类型：（1）在AML诊断时检查的多数骨髓中期细胞具有近四倍体核型；（2）骨髓中存在非常大的髓系原始细胞，以及红系和/或巨核细胞系的发育异常改变，提示AML起源于多能髓系祖细胞；（3）CD34抗原表达；（4）培养中粒细胞 - 巨噬细胞集落形成细胞生长低下；（5）存在白血病前期、较高年龄以及预后不良。
