title: Bone marrow cell cycle markers in inherited bone marrow failure syndromes.
abstract: Patients with inherited bone marrow failure syndromes (IBMFS) are at increased risk of acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS), possibly related to cell cycle dysregulation. In a cross-sectional analysis of bone marrow from 77 IBMFS, 71 sporadic conditions (AML, MDS, acquired aplastic anemia) and 22 normal controls we found overexpression of p53 in IBMFS, AML, and MDS; of Ki-67 in IBMFS and AML; and of survivin in IBMFS compared with all other groups. The patterns of expression of cell cycle markers in IBMFS are thus distinct. Longitudinal studies will determine the diagnostic and prognostic significance of these findings.
authors.affiliation: Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, Rockville, MD, USA.
authors.forename: Mohamad M
authors.initials: MM
authors.lastname: Al-Rahawan
authors.forename: Blanche P
authors.initials: BP
authors.lastname: Alter
authors.forename: Barbara J
authors.initials: BJ
authors.lastname: Bryant
authors.forename: M Tarek
authors.initials: MT
authors.lastname: Elghetany
mesh_terms: D015415:Biomarkers / Q000032:analysis; D001706:Biopsy; D001854:Bone Marrow Cells / Q000473:pathology*; D001855:Bone Marrow Diseases / Q000235:genetics / Q000473:pathology*; D002453:Cell Cycle; D005199:Fanconi Anemia / Q000235:genetics / Q000473:pathology; D006801:Humans; D051034:Inhibitor of Apoptosis Proteins; D019394:Ki-67 Antigen / Q000032:analysis; D015470:Leukemia, Myeloid, Acute / Q000235:genetics / Q000473:pathology*; D008869:Microtubule-Associated Proteins / Q000235:genetics; D009190:Myelodysplastic Syndromes / Q000235:genetics / Q000473:pathology*; D009363:Neoplasm Proteins / Q000235:genetics; D000077022:Survivin; D016159:Tumor Suppressor Protein p53 / Q000032:analysis
chemical_list: C107489:BIRC5 protein, human; D015415:Biomarkers; D051034:Inhibitor of Apoptosis Proteins; D019394:Ki-67 Antigen; D008869:Microtubule-Associated Proteins; D009363:Neoplasm Proteins; D000077022:Survivin; D016159:Tumor Suppressor Protein p53