Michiels J J, Adriaansen H J, Hagemeijer A, Hooijkaas H, van Dongen J J, Abels J
Department of Haematology, University Hospital Dijkzigt and Erasmus University, Rotterdam, The Netherlands.
Br J Haematol. 1988 Apr;68(4):423-6. doi: 10.1111/j.1365-2141.1988.tb04229.x.
The leukaemic cells in a 23-year-old man were small to medium-sized lymphoblasts with no cytoplasmic vacuoles and negative with PAS as well with peroxidase and acid phosphatase staining. Cytogenetic analysis showed -6, +12, -22, +mar (6p::22q), resulting in a trisomy 12 and monosomy of the long arm of chromosome 6. Immunological marker analysis revealed that the majority of the blasts was positive for terminal deoxynucleotidyl transferase (TdT) as well as surface membrane immunoglobulin (SmIg, mu, lambda), although B-ALL are supposed to be negative for TdT. The blasts were also positive for HLA-DR, CD9 (BA-2), CD10 (VIL-A1) and CD24 (BA-1), but negative for the B-cell markers CD20 (B1) and Y29/55. Double immunofluorescence staining confirmed that almost all TdT+ cells were also positive for Sm mu, Sm lambda, HLA-DR and CD10. We thus made a diagnosis of TdT+ B-ALL without Burkitt characteristics. Since we could not detect SmIg+/TdT+ cells in bone marrow samples from adult healthy volunteers and from 10 children with ALL in complete remission, we conclude that TdT+ B-ALL cells may not have a normal counterpart in bone marrow or represent a malignant counterpart of a very rare cell in an intermediate differentiation stage between the pre-B-cell and the early B lymphocyte.
一名23岁男性的白血病细胞为小至中等大小的淋巴母细胞，无细胞质空泡，糖原染色（PAS）、过氧化物酶染色及酸性磷酸酶染色均为阴性。细胞遗传学分析显示核型为-6，+12，-22，+mar（6p::22q），导致12号染色体三体及6号染色体长臂单体。免疫标志物分析显示，大多数母细胞末端脱氧核苷酸转移酶（TdT）及表面膜免疫球蛋白（SmIg，μ，λ）呈阳性，尽管B淋巴细胞白血病（B-ALL）通常TdT为阴性。母细胞HLA-DR、CD9（BA-2）、CD10（VIL-A1）及CD24（BA-1）也呈阳性，但B细胞标志物CD20（B1）及Y29/55为阴性。双重免疫荧光染色证实，几乎所有TdT阳性细胞Smμ、Smλ、HLA-DR及CD10也呈阳性。因此，我们诊断为无伯基特特征的TdT阳性B-ALL。由于在成年健康志愿者及10例完全缓解期的儿童ALL患者的骨髓样本中未检测到SmIg阳性/TdT阳性细胞，我们得出结论，TdT阳性B-ALL细胞在骨髓中可能没有正常对应物，或者代表前B细胞和早期B淋巴细胞之间一个非常罕见的中间分化阶段细胞的恶性对应物。
