Multilineage cell involvement in Ph1-negative, bcr-negative chronic myeloid leukemia

Leuk Res. 1988;12(8):637-45. doi: 10.1016/0145-2126(88)90097-5.

Abstract

We report a case of Ph1-negative, bcr-negative CML-BC, in which the primary leukemic cells displayed T-related antigens (CD7, CD4) in addition to HLA-DR and CD25 determinants. No B-lymphoid, myeloid and megakaryoblastic surface antigens were detected. In spite of this phenotype, DNA analysis revealed a germ-line configuration of the T-cell receptor beta chain gene region. Moreover, in-vitro culture studies demonstrated a proliferative response of the blast cell population to natural and recombinant myeloid-related factors, while no proliferative signal was observed in the presence of IL-2. The myeloid lineage was further demonstrated by the expression of myeloid-associated antigens on cultured blast cells, which still retained the CD7 antigen. Finally, cytogenetic analysis revealed a monosomy 7 which is usually associated with a stem cell leukemia. These results support the hypothesis that Ph1-negative, bcr-negative CML is characterized by the involvement of a multipotent stem cell capable of multilineage expression and indicate that differentiative and proliferative assays provide a further tool towards a more precise recognition of hematological disorders of uncertain origin.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Antigens, Differentiation, T-Lymphocyte / analysis
  • Biomarkers, Tumor / analysis
  • Cell Differentiation
  • Chromosome Aberrations / genetics
  • Chromosome Aberrations / pathology
  • Chromosome Disorders
  • Embryonal Carcinoma Stem Cells
  • Gene Rearrangement*
  • Humans
  • Leukemia, Myelogenous, Chronic, BCR-ABL Positive / genetics
  • Leukemia, Myelogenous, Chronic, BCR-ABL Positive / pathology*
  • Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative / pathology*
  • Male
  • Neoplastic Stem Cells / pathology*
  • Phenotype
  • Thymidine / metabolism
  • Tumor Stem Cell Assay

Substances

  • Antigens, Differentiation, T-Lymphocyte
  • Biomarkers, Tumor
  • Thymidine