D-2-hydroxyglutarate supports a tolerogenic phenotype with lowered major histocompatibility class II expression in non-malignant dendritic cells and acute myeloid leukemia cells

Haematologica. 2024 Aug 1;109(8):2500-2514. doi: 10.3324/haematol.2023.283597.

Abstract

D-2-hydroxyglutarate (D-2-HG) accumulates in patients with acute myeloid leukemia (AML) with mutated isocitrate dehydrogenase (IDH) and in other malignancies. D-2-HG suppresses antitumor T-cell immunity but little is known about potential effects on non-malignant myeloid cells. Here we show that D-2-HG impairs human but not murine dendritic cell differentiation, resulting in a tolerogenic phenotype with low major histocompatibility class II expression. In line with this, IDH-mutated AML blasts exhibited lower expression of HLA-DP and were less susceptible to lysis by HLA-DP-specific T cells. Interestingly, besides its expected impact on DNA demethylation, D-2-HG reprogrammed metabolism towards increased lactate production in dendritic cells and AML. Vitamin C accelerated DNA demethylation, but only the combination of vitamin C and glycolytic inhibition lowered lactate levels and supported major histocompatibility complex class II expression. Our results indicate an unexpected link between the immunosuppressive metabolites 2-HG and lactic acid and suggest a potentially novel therapeutic strategy with combinations of anti-glycolytic drugs and epigenetic modulators (hypomethylating agents) or other therapeutics for the treatment of AML.

Publication types

  • Research Support, Non-U.S. Gov't
  • Comment

MeSH terms

  • Animals
  • Cell Differentiation / drug effects
  • Dendritic Cells* / drug effects
  • Dendritic Cells* / immunology
  • Dendritic Cells* / metabolism
  • Glutarates* / metabolism
  • Glutarates* / pharmacology
  • Histocompatibility Antigens Class II* / genetics
  • Histocompatibility Antigens Class II* / metabolism
  • Humans
  • Immune Tolerance / drug effects
  • Isocitrate Dehydrogenase / genetics
  • Lactic Acid / metabolism
  • Leukemia, Myeloid, Acute* / drug therapy
  • Leukemia, Myeloid, Acute* / genetics
  • Leukemia, Myeloid, Acute* / immunology
  • Leukemia, Myeloid, Acute* / metabolism
  • Leukemia, Myeloid, Acute* / pathology
  • Mice
  • Phenotype

Substances

  • Glutarates
  • alpha-hydroxyglutarate
  • Histocompatibility Antigens Class II
  • Lactic Acid
  • Isocitrate Dehydrogenase

Grants and funding

Funding: This study was supported by funds from the Deutsche Forschungsgemeinschaft (DFG, German Research Foundation), project number 324392634 - TRR 221 (to ST and MK); DFG KFO 262 projects P9 and CP (to ST, MK, PJO, and KD) and Else Kröner-Fresenius Foundation project number 2018_A73 (to PJS).