Multilineage cell involvement in Ph1-negative, bcr-negative chronic myeloid leukemia.
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.
Callea
V
V
Divisione di Ematologia, Ospedali Riuniti di Reggio Calabria, Torino, Italy.
Morabito
F
F
Lista
P
P
Pegoraro
L
L
Cambrin
G R
GR
Guerrasio
A
A
Saglio
G
G
Tassinari
A
A
Fierro
M T
MT
Iacopino
P
P
D000368:Aged; D000945:Antigens, Differentiation, T-Lymphocyte / Q000032:analysis; D014408:Biomarkers, Tumor / Q000032:analysis; D002454:Cell Differentiation; D002869:Chromosome Aberrations / Q000235:genetics / Q000473:pathology; D025063:Chromosome Disorders; D054278:Embryonal Carcinoma Stem Cells; D015321:Gene Rearrangement; D006801:Humans; D015464:Leukemia, Myelogenous, Chronic, BCR-ABL Positive / Q000235:genetics / Q000473:pathology*; D054438:Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative / Q000473:pathology*; D008297:Male; D014411:Neoplastic Stem Cells / Q000473:pathology*; D010641:Phenotype; D013936:Thymidine / Q000378:metabolism; D014410:Tumor Stem Cell Assay
D000945:Antigens, Differentiation, T-Lymphocyte; D014408:Biomarkers, Tumor; D013936:Thymidine