Khalidi H S, Brynes R K, Medeiros L J, Chang K L, Slovak M L, Snyder D S, Arber D A
Division of Pathology, City of Hope National Medical Center, Duarte, California 91010, USA.
Mod Pathol. 1998 Dec;11(12):1211-21.
Immunophenotypic studies have a limited role in the diagnosis of chronic myelogenous leukemia (CML) but are increasingly being used in CML blast transformation (BT). Determination of the cell lineage of CML blasts is clinically important because patients with lymphoid blast transformation have a better response to chemotherapy and longer survival than those with other lineages. We studied the morphologic, cytochemical, immunophenotypic, cytogenetic, and molecular features of 20 patients with Philadelphia chromosome-positive CML and more than 10% blast cells in peripheral blood or bone marrow. The blasts were morphologically heterogeneous. CD33 was expressed in 19 cases (95%), followed by CD13 (85%), CD11c (80%), CD36 (60%), CD117 (40%), and CD15 (30%). Seven cases (35%) had a precursor-B lymphoid immunophenotype, and 13 (65%) had a predominantly myeloid immunophenotype. Of the former group, of which only one had a pure lymphoid phenotype, terminal deoxynucleotidyl transferase (TdT) and CD19 were expressed in 100%, CD10 in 85.7%, and CD20 in 14.3%. Of the latter group, all 13 expressed from 3 to 6 myeloid antigens, with 46.2% myeloperoxidase positive and 69.2% CD61 positive. No cases were interpreted as T lineage, but the T-cell antigens CD3, CD4, CD5, and CD7 were expressed in 5.0, 40.0, 5.3. and 30.0% of all cases, respectively. In most cases, the immunophenotype of the CML blasts could not be predicted from their morphologic features. Polymerase chain reaction showed that 80.0% of the lymphoid group and 37.5% of the myeloid group had immunoglobulin heavy-chain gene rearrangements. The frequent lineage infidelity of the blast cells in CML BT seems to be related to the stem cell origin of this disorder. Such lineage infidelity, however, makes classification of many cases difficult and the significance of and criteria for biphenotypic blast crisis of CML is yet to be determined.
免疫表型研究在慢性粒细胞白血病（CML）的诊断中作用有限，但在CML急变期（BT）中的应用日益增多。确定CML原始细胞的细胞系在临床上很重要，因为淋巴系急变的患者对化疗的反应更好，生存期比其他细胞系的患者更长。我们研究了20例费城染色体阳性CML且外周血或骨髓中原始细胞超过10%患者的形态学、细胞化学、免疫表型、细胞遗传学和分子特征。原始细胞在形态上具有异质性。19例（95%）表达CD33，其次是CD13（85%）、CD11c（80%）、CD36（60%）、CD117（40%）和CD15（30%）。7例（35%）具有前体B淋巴细胞免疫表型，13例（65%）主要具有髓系免疫表型。在前一组中，仅1例具有纯淋巴表型，末端脱氧核苷酸转移酶（TdT）和CD19在100%的病例中表达，CD10在85.7%的病例中表达，CD20在14.3%的病例中表达。在后一组中，所有13例表达3至6种髓系抗原，46.2%的病例髓过氧化物酶阳性，69.2%的病例CD61阳性。没有病例被判定为T细胞系，但T细胞抗原CD3、CD4、CD5和CD7分别在所有病例的5.0%、40.0%、5.3%和30.0%中表达。在大多数情况下，无法根据CML原始细胞的形态特征预测其免疫表型。聚合酶链反应显示，80.0%的淋巴系组和37.5%的髓系组有免疫球蛋白重链基因重排。CML急变期原始细胞频繁的系别不忠实似乎与该疾病的干细胞起源有关。然而，这种系别不忠实使得许多病例的分类困难，CML双表型急变期的意义和标准尚待确定。
