Identification of a myeloid committed progenitor as the cancer-initiating cell in acute promyelocytic leukemia

Blood. 2009 Dec 24;114(27):5415-25. doi: 10.1182/blood-2008-10-182071. Epub 2009 Oct 1.

Abstract

Acute promyelocytic leukemia (APL) is characterized by a block in differentiation and accumulation of promyelocytes in the bone marrow and blood. The majority of APL patients harbor the t(15:17) translocation leading to expression of the fusion protein promyelocytic-retinoic acid receptor alpha. Treatment with retinoic acid leads to degradation of promyelocytic-retinoic acid receptor alpha protein and disappearance of leukemic cells; however, 30% of APL patients relapse after treatment. One potential mechanism for relapse is the persistence of cancer "stem" cells in hematopoietic organs after treatment. Using a novel sorting strategy we developed to isolate murine myeloid cells at distinct stages of differentiation, we identified a population of committed myeloid cells (CD34(+), c-kit(+), FcgammaRIII/II(+), Gr1(int)) that accumulates in the spleen and bone marrow in a murine model of APL. We observed that these cells are capable of efficiently generating leukemia in recipient mice, demonstrating that this population represents the APL cancer-initiating cell. These cells down-regulate the transcription factor CCAAT/enhancer binding protein alpha (C/EBPalpha) possibly through a methylation-dependent mechanism, indicating that C/EBPalpha deregulation contributes to transformation of APL cancer-initiating cells. Our findings provide further understanding of the biology of APL by demonstrating that a committed transformed progenitor can initiate and propagate the disease.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Antigens, CD34 / metabolism
  • Bone Marrow Cells / metabolism
  • Bone Marrow Cells / pathology*
  • CCAAT-Enhancer-Binding Protein-alpha / genetics
  • Calgranulin A / genetics
  • Calgranulin A / metabolism
  • Cell Line, Tumor
  • DNA Methylation
  • Female
  • Flow Cytometry
  • Humans
  • Kaplan-Meier Estimate
  • Leukemia, Promyelocytic, Acute / blood
  • Leukemia, Promyelocytic, Acute / genetics
  • Leukemia, Promyelocytic, Acute / pathology*
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Transgenic
  • Myeloid Cells / metabolism
  • Myeloid Cells / pathology*
  • Neoplastic Stem Cells / metabolism
  • Neoplastic Stem Cells / pathology*
  • Oncogene Proteins, Fusion / genetics
  • Oncogene Proteins, Fusion / metabolism
  • Promoter Regions, Genetic / genetics
  • Proto-Oncogene Proteins c-kit / metabolism
  • U937 Cells

Substances

  • Antigens, CD34
  • CCAAT-Enhancer-Binding Protein-alpha
  • Calgranulin A
  • Oncogene Proteins, Fusion
  • promyelocytic leukemia-retinoic acid receptor alpha fusion oncoprotein
  • Proto-Oncogene Proteins c-kit