Clinical and laboratory characteristics of acute leukemia with the 4;11 translocation.
This report describes the clinical and laboratory features of seven cases of acute leukemia associated with the 4;11 chromosomal translocation. All seven children had acute lymphoblastic leukemia by standard morphologic and cytochemical criteria. Leukemic blasts from six of seven patients were terminal deoxynucleotidyl transferase-positive. Immunologic phenotyping suggested the leukemias were of B cell origin; blasts from five patients expressed HLA-DR and p24 (CD-9 antibody), blasts from three patients expressed B4 (CD-19), and blasts from two patients expressed the common acute lymphoblastic leukemia antigen (CD-10). One patient's leukemic blasts contained cytoplasmic immunoglobulin. Analysis of DNA from four of five patients demonstrated additional evidence of B cell differentiation with heavy-chain immunoglobulin gene rearrangement. When DNA from the four patients with heavy-chain immunoglobulin gene rearrangement was analyzed, one patient's DNA demonstrated light-chain immunoglobulin gene rearrangement. However, flow cytometric analysis of blasts from three patients showed the simultaneous expression of the lymphoid-associated antigen B4 (CD-19) and the myeloid-associated antigen My-1 (X-Hapten). Electron microscopic examination of blasts from one patient that expressed both lymphoid- and myeloid-associated antigens demonstrated ultrastructural characteristics of both lineages. These findings suggest that acute leukemia with the t(4;11) abnormality has mixed lineage characteristics as a result of leukemogenesis in a multipotential progenitor cell or aberrant gene expression later in differentiation. Furthermore, serial analysis of karyotype, immunophenotype, and heavy-chain immunoglobulin genes revealed changes in these biologic markers over time, suggesting continued chromosome rearrangement and gene modulation after the leukemogenic event in cells with the t(4;11).
D000208:Acute Disease; D000293:Adolescent; D000954:Antigens, Surface / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D002905:Chromosomes, Human, 4-5; D002906:Chromosomes, Human, 6-12 and X; D004247:DNA / Q000032:analysis; D004253:DNA Nucleotidylexotransferase / Q000032:analysis; D005260:Female; D006801:Humans; D007136:Immunoglobulins / Q000235:genetics; D007223:Infant; D007621:Karyotyping; D007938:Leukemia / Q000235:genetics* / Q000276:immunology / Q000473:pathology; D008297:Male; D009857:Oncogenes; D011995:Recombination, Genetic; D014178:Translocation, Genetic
D000954:Antigens, Surface; D007136:Immunoglobulins; D004247:DNA; D004253:DNA Nucleotidylexotransferase