title: [Exacerbation of acute leukemia bearing isolated i(17q) along with proliferation of blasts with high BMI-1 expression].
abstract: We report a case of acute leukemia with an isolated isochromosome 17q karyotypic abnormality, which may be transformed from myeloproliferative disease (MPD)/myelodysplastic syndrome (MDS). A 69-year-old male patient with 27% of blasts in the peripheral blood underwent hematological examinations including cytochemical staining of cells such as myeloperoxydase (MPO), surface marker study on blasts, chromosomal test and bcr-abl mRNA analysis. The cytological and molecular findings (MPO-positive, myeloid marker CD13 expression (67.3%) and megakaryocytic marker CD41 expression (24.8%)) indicated that the blasts were consistent with myeloid leukemic cells partially committed to megakaryocytes. He was diagnosed as having leukemic transformation from MPD/MDS based on history of leukocytosis and thrombocytosis, isolated i(17q), bcr-abl negative, hepatosplenomegaly, increased eosinophil/basophil count and cytologic dysplasia. Positivity of BMI-1 in CD34+ blasts was 25.8% at the diagnosis and anti-leukemic drugs including anthracyclines were effective for his disease control during 6 months. However, the CD34+ cells turned out to highly express BMI-1 (83.1%), and leukemic cells started to increase progressively following which the leukemic cells failed to respond efficiently to any anti-leukemic drugs. Thus, expression of BMI-1 was well correlated with the disease progression, growth ability of blasts and resistance to anti-cancer drugs, indicating that BMI-1 positivity in CD34+blasts is an excellent molecular marker for disease progression and prognosis in such patients.
authors.affiliation: Department of Hematology and Oncology, Research Institute for Radiation Biology and Medicine, Hiroshima University.
authors.forename: Keichiro
authors.initials: K
authors.lastname: Mihara
authors.forename: Miki
authors.initials: M
authors.lastname: Kido
authors.forename: Nanae
authors.initials: N
authors.lastname: Nakaju
authors.forename: Sachiko
authors.initials: S
authors.lastname: Fukumoto
authors.forename: Ryoko
authors.initials: R
authors.lastname: Matsumoto
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Takihara
authors.forename: Akiro
authors.initials: A
authors.lastname: Kimura
mesh_terms: D000208:Acute Disease; D000368:Aged; D002886:Chromosomes, Human, Pair 17 / Q000235:genetics*; D018450:Disease Progression; D006801:Humans; D018404:Isochromosomes / Q000235:genetics*; D007938:Leukemia / Q000235:genetics*; D008297:Male; D009687:Nuclear Proteins / Q000032:analysis*; D063150:Polycomb Repressive Complex 1; D011518:Proto-Oncogene Proteins / Q000032:analysis*; D012097:Repressor Proteins / Q000032:analysis*
chemical_list: C069077:BMI1 protein, human; D009687:Nuclear Proteins; D011518:Proto-Oncogene Proteins; D012097:Repressor Proteins; D063150:Polycomb Repressive Complex 1