Menin inhibition enhances graft-versus-leukemia effects by T-cell activation and endogenous retrovirus induction in AML

V Viktor Fetsch (University Medical Center Freiburg, Freiburg, Germany) L Lennard Schwöbel (1Department of Medicine I, Faculty of Medicine, University Medical Center, University of Freiburg, Freiburg, Germany) E Ezgi Özyerli-Göknar A Anna-Verena Stell (1Department of Internal Medicine I, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany) M Marco Punta (5Unit of Immunogenetics, Leukemia Genomics and Immunobiology, Istituto di Ricovero e Cura a Carattere Scientifico, San Raffaele Scientific Institute, Milan, Italy) T Thomas Plenge (Hannover Medical School, Hannover, Germany) T Tabea Klaus (7Hematology, Hemostasis, Oncology, and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany) M Manoj K. Gupta (Hannover Medical School, Hannover, Germany) G Geoffroy Andrieux K Khalid Shoumariyeh (1Department of Medicine I, Faculty of Medicine, University Medical Center, University of Freiburg, Freiburg, Germany) S Sophie Pfeiffer (1Department of Medicine I, Faculty of Medicine, University Medical Center, University of Freiburg, Freiburg, Germany) E Eyleen Corrales (8Institute of Medical Bioinformatics and Systems Medicine, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany) L Lina Schlenke (1Department of Medicine I, Faculty of Medicine, University Medical Center, University of Freiburg, Freiburg, Germany) H Hosna Baniadam (1Department of Medicine I, Faculty of Medicine, University Medical Center, University of Freiburg, Freiburg, Germany) S Simon M. Brandl (2Faculty of Biology, Albert-Ludwigs-University, Freiburg, Germany) M Massimo Andreis (12Department of Biomedicine, University Hospital Basel and University of Basel, Basel, Switzerland) M Michal Remen (12Department of Biomedicine, University Hospital Basel and University of Basel, Basel, Switzerland) A Alina Hartmann (University Medical Center Freiburg, Freiburg, Germany) K Kathleen Grueter (13German Cancer Research Center, Heidelberg, Germany) M Melissa Zwick (Medical Center-University of Freiburg) N Natalie Köhler M Monika Kuban (13German Cancer Research Center, Heidelberg, Germany) E Eric Metzger C Christoph Rummelt (1Department of Medicine I, Faculty of Medicine, University Medical Center, University of Freiburg, Freiburg, Germany) J Justus Duyster M Melanie Boerries M Maike Hofmann J Julian Färber (17Department of Pediatrics, University Medical Center Freiburg, Freiburg, Germany) L Lukas M. Braun A Alexander Zähringer (1Department of Medicine I, Faculty of Medicine, University Medical Center, University of Freiburg, Freiburg, Germany) M Michael Lübbert (10Department of Medicine I, Medical Center–University of Freiburg, Faculty of Medicine, University of Freiburg, Germany) C Cristina Toffalori (IRCCS San Raffaele Scientific Institute, Milano, Italy) L Luca Vago (Vita-Salute San Raffaele University, Italy) F Florian H. Heidel S Susana Minguet (2Faculty of Biology, Albert-Ludwigs-University, Freiburg, Germany) P Petya Apostolova (12Department of Biomedicine, University Hospital Basel and University of Basel, Basel, Switzerland) T Tobias Feuchtinger K Kristina Maas-Bauer (1Department of Internal Medicine I, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany) F Franziska Blaeschke (13German Cancer Research Center, Heidelberg, Germany) M Michael W. M. Kühn (1Department of Hematology and Oncology, Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany) H H. T. Marc Timmers T Tobias Wertheimer F Florian Perner R Robert Zeiser

Abstract

Abstract Acute myeloid leukemia (AML) carrying chromosomal rearrangements involving the lysine methyltransferase 2A (KMT2A) gene frequently relapse after allogeneic hematopoietic cell transplant (allo-HCT). Pharmacological blockade of the menin-KMT2A interaction disrupts the assembly of oncogenic KMT2A complexes on chromatin, thereby attenuating aberrant self-renewal and inducing myeloid differentiation. We found that beyond this antileukemic mechanism, menin inhibition induced class II transactivator and major histocompatibility complex II (MHC-II) expression in KMT2A-rearranged and NPM1-mutated AML cells in vitro and in vivo. Increased MHC-II expression sensitized AML cells to T-cell–mediated elimination after allo-HCT in mice. Menin inhibition also increased MHC-II expression on primary human AML cells, and enhanced the graft-versus-leukemia (GVL) effect in human xenograft models. Mechanistically, menin inhibition increased expression of multiple human endogenous retroviruses (HERV), leading to consecutive interferon-stimulated gene upregulation and enhanced MHC-II expression. Additionally, menin inhibition directly promoted antitumor effector functions of donor T cells, causing increased tumor necrosis factor-alfa, interferon-gamma, perforin, and granzyme A/B production and cytolytic activity. T-cell exhaustion and menin-KMT2A binding to genes encoding for negative regulators of T-cell activation were reduced by menin inhibition. These findings indicate that menin inhibition enhances the GVL effect via the HERV/MHC-II axis in AML cells and promotes cytotoxicity of donor T cells, which provides a rationale for a clinical trial using menin inhibition as maintenance after allo-HCT.

Article Details

Journal Blood
Volume / Issue Vol. 147, Issue 5
Published January 29, 2026
Pages 584-601
ISSN 0006-4971
Publisher Elsevier BV

Journal Info

Blood

Elsevier BV

ISSN: 0006-4971 Health Sciences

Authors (44)

V

Viktor Fetsch

University Medical Center Freiburg, Freiburg, Germany

L

Lennard Schwöbel

1Department of Medicine I, Faculty of Medicine, University Medical Center, University of Freiburg, Freiburg, Germany

E

Ezgi Özyerli-Göknar

A

Anna-Verena Stell

1Department of Internal Medicine I, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany

M

Marco Punta

5Unit of Immunogenetics, Leukemia Genomics and Immunobiology, Istituto di Ricovero e Cura a Carattere Scientifico, San Raffaele Scientific Institute, Milan, Italy

T

Thomas Plenge

Hannover Medical School, Hannover, Germany

T

Tabea Klaus

7Hematology, Hemostasis, Oncology, and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany

M

Manoj K. Gupta

Hannover Medical School, Hannover, Germany

G

Geoffroy Andrieux

K

Khalid Shoumariyeh

1Department of Medicine I, Faculty of Medicine, University Medical Center, University of Freiburg, Freiburg, Germany

S

Sophie Pfeiffer

1Department of Medicine I, Faculty of Medicine, University Medical Center, University of Freiburg, Freiburg, Germany

E

Eyleen Corrales

8Institute of Medical Bioinformatics and Systems Medicine, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany

L

Lina Schlenke

1Department of Medicine I, Faculty of Medicine, University Medical Center, University of Freiburg, Freiburg, Germany

H

Hosna Baniadam

1Department of Medicine I, Faculty of Medicine, University Medical Center, University of Freiburg, Freiburg, Germany

S

Simon M. Brandl

2Faculty of Biology, Albert-Ludwigs-University, Freiburg, Germany

M

Massimo Andreis

12Department of Biomedicine, University Hospital Basel and University of Basel, Basel, Switzerland

M

Michal Remen

12Department of Biomedicine, University Hospital Basel and University of Basel, Basel, Switzerland

A

Alina Hartmann

University Medical Center Freiburg, Freiburg, Germany

K

Kathleen Grueter

13German Cancer Research Center, Heidelberg, Germany

M

Melissa Zwick

Medical Center-University of Freiburg

N

Natalie Köhler

M

Monika Kuban

13German Cancer Research Center, Heidelberg, Germany

E

Eric Metzger

C

Christoph Rummelt

1Department of Medicine I, Faculty of Medicine, University Medical Center, University of Freiburg, Freiburg, Germany

J

Justus Duyster

M

Melanie Boerries

M

Maike Hofmann

J

Julian Färber

17Department of Pediatrics, University Medical Center Freiburg, Freiburg, Germany

L

Lukas M. Braun

A

Alexander Zähringer

1Department of Medicine I, Faculty of Medicine, University Medical Center, University of Freiburg, Freiburg, Germany

M

Michael Lübbert

10Department of Medicine I, Medical Center–University of Freiburg, Faculty of Medicine, University of Freiburg, Germany

C

Cristina Toffalori

IRCCS San Raffaele Scientific Institute, Milano, Italy

L

Luca Vago

Vita-Salute San Raffaele University, Italy

F

Florian H. Heidel

S

Susana Minguet

2Faculty of Biology, Albert-Ludwigs-University, Freiburg, Germany

P

Petya Apostolova

12Department of Biomedicine, University Hospital Basel and University of Basel, Basel, Switzerland

T

Tobias Feuchtinger

K

Kristina Maas-Bauer

1Department of Internal Medicine I, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany

F

Franziska Blaeschke

13German Cancer Research Center, Heidelberg, Germany

M

Michael W. M. Kühn

1Department of Hematology and Oncology, Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany

H

H. T. Marc Timmers

T

Tobias Wertheimer

F

Florian Perner

R

Robert Zeiser