HSCs/MPPs as cells of origin with altered differentiation hierarchy impairing immunomicroenvironment in PML::RARA and CBFα/β fusion AML

Proc Natl Acad Sci U S A. 2026 Feb 10;123(6):e2526334123. doi: 10.1073/pnas.2526334123. Epub 2026 Feb 6.

Abstract

The theoretical possibility for leukemia stem cells (LSCs) to produce both leukemia blasts and dysfunctional immune cells remains underexplored. Here, we investigate three major fusion transcription factor (fTF)-driven acute myeloid leukemia (AML) subtypes [RUNX1(CBFα)::RUNX1T1, PML::RARA, and CBFB::MYH11] using two optimized single-cell RNA-sequencing technologies to trace fTF expression in 24 de novo AML patients. We demonstrate that the fTFs are widely expressed not only in leukemia blasts but also in differentiated myeloid and lymphoid cells, indicating hematopoietic stem cells or multipotent progenitors (HSCs/MPPs) as LSCs that propagate altered cellular differentiation hierarchies, including immune cells. DNA-FISH confirms the presence of fTFs in T lymphoid and erythroid cells, and targeted sequencing of secondary mutations in sublineages of cells corroborates hierarchical and stepwise leukemogenesis. By tracking RUNX1::RUNX1T1-expressing cells in patients with or without relapse post-frontline chemotherapy, we highlight the necessity of eradicating LSCs to achieve sustained long-term complete remission and restore a functional immune system capable of suppressing residual disease over time. Comparative single-cell transcriptome analyses further reveal that fTFs are associated with AML subtype-specific differentiation defects in both innate and adaptive immune compartments, suggesting an altered landscape of immune cell-cell communication networks that may facilitate the survival and proliferation of leukemic blasts. Through the examination of intercellular communications among various putative fTF+ and normal cell populations, we developed a ligand-receptor (L-R)-based risk-scoring model with independent prognostic value. Collectively, these findings provide insights into the cells of origin of LSCs and the implications of fTF expression for the immune landscape of AML.

Keywords: acute myeloid leukemia; fusion transcription factor; leukemia stem cell; single-cell RNA sequencing; subtype-specific characteristics.

MeSH terms

  • Cell Differentiation / genetics
  • Core Binding Factor Alpha 2 Subunit / genetics
  • Core Binding Factor Alpha 2 Subunit / metabolism
  • Core Binding Factor beta Subunit / genetics
  • Core Binding Factor beta Subunit / metabolism
  • Female
  • Hematopoietic Stem Cells* / immunology
  • Hematopoietic Stem Cells* / metabolism
  • Hematopoietic Stem Cells* / pathology
  • Humans
  • Leukemia, Myeloid, Acute* / genetics
  • Leukemia, Myeloid, Acute* / immunology
  • Leukemia, Myeloid, Acute* / pathology
  • Male
  • Neoplastic Stem Cells* / immunology
  • Neoplastic Stem Cells* / metabolism
  • Neoplastic Stem Cells* / pathology
  • Oncogene Proteins, Fusion* / genetics
  • Oncogene Proteins, Fusion* / metabolism
  • RUNX1 Translocation Partner 1 Protein / genetics
  • RUNX1 Translocation Partner 1 Protein / metabolism
  • Tumor Microenvironment / immunology

Substances

  • Oncogene Proteins, Fusion
  • Core Binding Factor Alpha 2 Subunit
  • promyelocytic leukemia-retinoic acid receptor alpha fusion oncoprotein
  • RUNX1 Translocation Partner 1 Protein
  • Core Binding Factor beta Subunit
  • RUNX1 protein, human
  • RUNX1T1 protein, human