title: Leukemic transformation of primary myelofibrosis: immunophenotype, genotype and growth characteristics of blast cells.
abstract: Blast cells from six patients with leukemic transformation of primary myelofibrosis (PMF) were studied by morphology, immunophenotype and genotype as well as response to hematopoietic growth factors. The majority of the patients showed granulocytic or granulo-monocytic blasts, and only one had T lymphoid-monocytic blasts. None of the patients showed rearrangement of Ig or TCR genes, or the existence of the bcr-abl fused gene. A prominent growth response to GM-CSF and IL-3 was evident in all of the patients examined in liquid as well as semisolid cultures. The response to G-CSF was observed in four of the six patients in suspension culture, and in two of three patients in the clonogenic assay. Stem cell factor (SCF) was a weak growth stimulant, however the combination of this factor with GM-CSF or IL-3 was synergistically stimulatory. These results suggest that leukemic transformation of PMF occurs mainly at the level of myeloid stem cell, and that GM-CSF, IL-3, G-CSF and SCF are major growth factors for the blast cells in these cases.
authors.affiliation: Department of Environment and Mutation, Hiroshima University, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Kimura
authors.forename: K
authors.initials: K
authors.lastname: Kawaishi
authors.forename: Y
authors.initials: Y
authors.lastname: Nakata
authors.forename: H
authors.initials: H
authors.lastname: Hyodo
authors.forename: A
authors.initials: A
authors.lastname: Kuramoto
authors.forename: Y
authors.initials: Y
authors.lastname: Satow
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000032:analysis; D000951:Antigens, Neoplasm / Q000032:analysis; D001483:Base Sequence; D014408:Biomarkers, Tumor / Q000032:analysis; D002455:Cell Division / Q000187:drug effects; D002471:Cell Transformation, Neoplastic / Q000235:genetics; D004273:DNA, Neoplasm / Q000235:genetics; D018450:Disease Progression; D004357:Drug Synergism; D005260:Female; D005838:Genotype; D016178:Granulocyte-Macrophage Colony-Stimulating Factor / Q000494:pharmacology; D006801:Humans; D016130:Immunophenotyping; D007377:Interleukin-3 / Q000494:pharmacology; D007951:Leukemia, Myeloid / Q000235:genetics / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D008969:Molecular Sequence Data; D014411:Neoplastic Stem Cells / Q000187:drug effects / Q000473:pathology; D055728:Primary Myelofibrosis / Q000235:genetics / Q000473:pathology*; D019089:Stem Cell Factor / Q000494:pharmacology; D014407:Tumor Cells, Cultured / Q000187:drug effects
chemical_list: D015703:Antigens, CD; D000951:Antigens, Neoplasm; D014408:Biomarkers, Tumor; D004273:DNA, Neoplasm; D007377:Interleukin-3; D019089:Stem Cell Factor; D016178:Granulocyte-Macrophage Colony-Stimulating Factor