Incidence of preleukemic fusion genes in healthy subjects

Neoplasma. 2016;63(5):659-72. doi: 10.4149/neo_2016_502.

Abstract

The diagnostics of leukemia relies upon multi-parametric approach involving a number of different pathology disciplines such as flow cytometry, histopathology, cytogenetics and molecular genetics [fluorescent in situ hybridization (FISH) and polymerase chain reaction (PCR)]. Childhood leukemia is often determined by the presence of specific chromosomal translocation that entails the generation of preleukemic fusion genes (PFG). In the last two decades, several studies have reported observations that PFG are present in healthy population and not necessarily result in leukemia. The first such study by Limpens and colleagues on t(14/18)/ BCL2-JH [1] and next in line [2, 3] led to many questions regarding the significance of these chromosomal translocations in leukemogenesis. However, the data on the incidence of PFG are contradictive. This review aims to highlight the molecular genetic approaches used by various studies with regard to differences in diagnostics and incidence of PFG in healthy subjects. The focus is on the incidence and prevalence of the most common PFG such as TEL-AML1, MLL-AF4, BCR-ABL (p190), AML1-ETO, PML-RARA, and CBFB-MYH11 detected in umbilical cord blood, in neonatal blood spots (Guthrie cards (GC)), bone marrow, peripheral blood and tissues of amortized fetuses. We conclude that the incidence of PFG is significantly higher than incidence of leukemia and more sophisticated analysis of PFG in leukemogenic cell populations is warranted to relate the occurrence of PFG with leukemia. The emerging notion is that only those PFG may contribute to development of leukemia which arise in stem cells at specific time windows during development. Thus, screening of PFG in subpopulations of stem cells may be a challenge for assessment of predisposition to leukemia and for validation of cell transplant to minimize donor cell-derived leukemia.

Keywords: childhood leukemia; preleukemic fusion genes; stem cells..

Publication types

  • Review

MeSH terms

  • Core Binding Factor Alpha 2 Subunit / blood
  • Core Binding Factor Alpha 2 Subunit / genetics
  • Fetal Blood
  • Fusion Proteins, bcr-abl / blood
  • Fusion Proteins, bcr-abl / genetics
  • Humans
  • Leukemia, Myeloid, Acute / diagnosis*
  • Leukemia, Myeloid, Acute / genetics*
  • Mass Screening / methods
  • Myeloid-Lymphoid Leukemia Protein / blood
  • Myeloid-Lymphoid Leukemia Protein / genetics
  • Oncogene Proteins, Fusion / blood*
  • Oncogene Proteins, Fusion / genetics*
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / diagnosis*
  • Precursor Cell Lymphoblastic Leukemia-Lymphoma / genetics*
  • RUNX1 Translocation Partner 1 Protein / blood
  • RUNX1 Translocation Partner 1 Protein / genetics

Substances

  • AML1-ETO fusion protein, human
  • CBFbeta-MYH11 fusion protein
  • Core Binding Factor Alpha 2 Subunit
  • MLL-AF4 fusion protein, human
  • Oncogene Proteins, Fusion
  • PML-RARa bcr1 fusion protein, human
  • RUNX1 Translocation Partner 1 Protein
  • TEL-AML1 fusion protein
  • Myeloid-Lymphoid Leukemia Protein
  • Fusion Proteins, bcr-abl