bims-tremyl Biomed News
on Therapy resistance biology in myeloid leukemia
Issue of 2026–03–15
thirty-one papers selected by
Paolo Gallipoli, Barts Cancer Institute, Queen Mary University of London



  1. Leukemia. 2026 Mar 11.
      Menin inhibition leads to an antileukemic effect through hematopoietic differentiation. Treatment with the menin inhibitor revumenib results in clinical remissions in relapsed or refractory (R/R) acute myeloid leukemia (AML) with either rearrangement of lysine methyltransferase 2A (KMT2A) or mutation in nucleophosmin 1 (NPM1), leading to regulatory approval of this drug. However, determinants of response to revumenib have not been fully elucidated. We examined the immunophenotype of leukemia cells by flow cytometry, in sequential bone marrow specimens from 48 patients with R/R AML treated with revumenib. We observed dynamic changes in the immunophenotype after treatment in 16 of 31 (52%) patients, characterized by a switch from a myeloid/stem-like to a monocytic or myelomonocytic immunophenotype, or vice versa, or by substantial changes in the intensity of antigen expression or in patterns of leukemia-associated immunophenotypes. Morphologic remission with undetectable measurable residual disease (MRD) by flow cytometry following revumenib was associated with improved overall survival, with a median of 23.6 months compared with 20.8 months in patients with morphologic response and detectable MRD, and 3.2 months in non-responders. In summary, treatment monitoring of AML by flow cytometry, following menin inhibition, requires recognition of phenotypic changes associated with differentiation.
    DOI:  https://doi.org/10.1038/s41375-026-02905-6
  2. Leukemia. 2026 Mar 09.
      Whether mitigation of myeloproliferation improves prognosis of CMML independently of bone marrow response is unknown. Flow-defined classical monocytes (cMo) and immature granulocytes (iGRAN) have not yet been studied as biomarkers of response. We inspected the prognostic value of WBC, circulating monocytes, cMo and iGRANs in the 120 DACOTA (NCT02214407) patients randomized to decitabine (n = 63) or hydroxyurea (n = 57) evaluated after 3 cycles with BM aspiration and complete blood count. Across arms, 59% and 56% patients had monocytes > 1 × 109/L or WBC > 10 × 109/L at the 3- and 6-cycle evaluation respectively. After 6 cycles, persistence of monocytes > 1 × 109/L or WBC > 10 × 109/L increased the hazard of death (HR = 5.38, p = 0.0003) irrespective of treatment, baseline CPSS and persistence of BM blast excess. After 3 cycles, both higher absolute cMo and iGRAN counts independently predicted poorer OS, without significant interaction with treatment arm. Median OS from landmark was 35.1 months in the 28% patients with cMo ≤ 0.94 ×109/L AND iGRAN ≤ 0.40 ×109/L versus 15.3 months in others (p = 0.013). Biomarkers integrating blood counts and flow cytometry may predict CMML prognosis irrespective of treatment.
    DOI:  https://doi.org/10.1038/s41375-026-02901-w
  3. Blood. 2026 Mar 09. pii: blood.2025031883. [Epub ahead of print]
      The terms clonal monocytosis of undetermined significance (CMUS) and clonal cytopenia and monocytosis of undetermined significance (CCMUS) were introduced by the International Consensus Classification of Myeloid Neoplasms (ICC) to describe cases of clonal hematopoiesis (CH) and a concurrent monocytosis, that did not meet the diagnostic criteria of chronic myelomonocytic leukemia (CMML). To date, their practical relevance as clinicopathological entities at a population level has not been assessed. Here, we assess the prevalence, significance and natural history of CMUS and CCMUS amongst 431,531 UK Biobank participants through analysis of clinical, genomic and health outcome data. We find that CMUS with an absolute monocytosis and CCMUS are high-risk entities strongly associated with incident myeloid neoplasia (MN), cardiovascular and renal disease. Noting the overall higher monocyte counts in men and the low rate of progression of DNMT3A-CMUS, we show that amending the definition of CMUS/CCMUS to incorporate sex-specific monocyte thresholds and the exclusion of isolated DNMT3A mutations from the definition significantly strengthens the association with incident MN. Finally, given their association with poor outcomes, we develop MoSAIC, a machine learning classifier to infer the presence of SRSF2 mutations (associated with high MN risk) amongst individuals with monocytosis, based on complete blood count indices alone. We corroborate our findings in an independent cohort of 625,328 Danish primary care patients. Our findings underscore the clinical relevance of CMUS and CCMUS as distinct high-risk states within the spectrum of clonal hematopoiesis, and establish an evidence base to refine their diagnostic definition.
    DOI:  https://doi.org/10.1182/blood.2025031883
  4. Nat Genet. 2026 Mar;58(3): 582-592
      Therapy-related myeloid neoplasm (tMN) is a fatal consequence of exposure to cytotoxic therapy administered in the treatment of cancer. Individuals with pre-existing TP53 clonal hematopoiesis (CH) are at high risk of tMN, with avoidance of therapy being the only strategy to reduce tMN risk. Here, in four randomized clinical trials, we show that the CDK4/6 inhibitor trilaciclib, given in conjunction with a variety of chemotherapeutic regimens and across diverse populations of patients with cancer, mitigates chemotherapy-related expansion of CH clones with mutations in DNA damage response genes, including TP53. This finding was also observed in a syngeneic mouse model of TP53-mutant CH, demonstrating that CDK4/6 inhibition blocks platinum-induced TP53 competitive repopulation through promoting hematopoietic stem and progenitor quiescence and decreasing the stemness advantage of TP53-mutant clones. This represents a proof of concept for a potential pharmacologic strategy to block chemotherapy-induced expansion of preleukemic TP53-mutant clones.
    DOI:  https://doi.org/10.1038/s41588-026-02526-w
  5. Br J Haematol. 2026 Mar 08.
      The modern management of chronic myeloid leukaemia (CML) identifies a new therapeutic goal of treatment-free remission (TFR). Half of CML patients in durable deep molecular response (DMR) (MR4 or better) can remain off tyrosine kinase inhibitors (TKIs) without experiencing loss of major molecular response. Despite a large number of TFR studies to date, there are no consistent predictors of successful TFR. We conducted a single-centre cohort study on 197 patients discontinuing TKIs in DMR ≥1 year and TKI therapy ≥3 years. After TKI discontinuation, 98 patients (49.7%) lost MR4; of these, 90 (91.8%; and 45.7% of the whole cohort) lost major molecular response (MMR or MR3) after a median of 3.8 months (1-93.3). The 2-year probability of TFR (pTFR) was 57.7%. In multivariable analysis, male sex, age at diagnosis >40 years, faster achievement of MR4 and longer duration of DMR were the only variables significantly associated with higher pTFR. Based on the multivariable analysis results, we built a TFR prognostic score (TPS) able to distinguish three groups with different 2-year pTFR: good (89.9%), intermediate (61.2%) and poor (18.4%) TFR probability (p < 0.0001). We validated the TPS on an independent cohort of 91 patients. We propose that the TPS could become a useful guide for CML clinicians.
    Keywords:   DMR ; TFR ; TKI ; prognostic score
    DOI:  https://doi.org/10.1111/bjh.70409
  6. Leukemia. 2026 Mar 09.
      Disease progression, graft-versus-host disease (GVHD), and non-relapse mortality (NRM) are the main causes of failure after allogeneic hematopoietic cell transplantation (SCT) for myeloid neoplasms. T-cell epitope-based models classify HLA-DPB1 mismatches by permissiveness and have been associated with differential risks of GVHD, relapse, and NRM. However, most studies were conducted before the routine use of post-transplant cyclophosphamide (PTCy) as GVHD prophylaxis for unrelated donor (UD) SCT. We retrospectively analyzed 541 adults undergoing 8/8 UD SCT with PTCy prophylaxis, categorized as DP-matched (DP-M, n = 176), permissive mismatch (DP-P, n = 219), non-permissive graft-versus-host (DP-NP-GVH, n = 82), or host-versus-graft (DP-NP-HVG, n = 64). Outcomes were compared across groups with stratification by disease risk and remission/MRD status. Two-year relapse incidence was lower with DP-P versus DP-M (18% vs 28%; HR 0.6, 95% CI 0.4-0.9, p = 0.03), most pronounced in high-risk AML/MDS, where relapse rates approached those of lower-risk disease. This effect persisted after adjustment for remission and MRD status. GVHD incidence was unaffected by DPB1 status. OS and PFS did not differ significantly; age and comorbidity were dominant predictors of NRM. In UD SCT with PTCy, DPB1-permissive mismatching reduces relapse in high-risk AML/MDS without increasing GVHD or NRM, supporting DP-P mismatching as an actionable donor-selection criterion.
    DOI:  https://doi.org/10.1038/s41375-026-02907-4
  7. Am J Hematol. 2026 Mar 08.
      Preclinical studies showed a synergistic antileukemia activity with combination of selective XPO1 inhibitor selinexor (SEL) and venetoclax (VEN), with potential to overcome VEN resistance by reducing the anti-apoptotic protein MCL1. In an investigator-sponsored, open-label, phase Ib study (NCT03955783), adult patients with relapsed or refractory acute myeloid leukemia (R/R AML) were enrolled. After the dose-escalation phase, SEL 80 mg po weekly plus VEN 400 mg/day following ramp-up was deemed the recommended phase II dose. Responses were assessed with IWG2003 and ELN 2022 criteria. Nineteen patients with R/R AML were enrolled. Median age at enrollment was 67.2 (range, 21.1-83.8) years. Overall, patients received median of 3 (range, 1-5) prior lines of therapy. The most common grade 3-5 treatment emergent adverse events (TEAE) were anemia (39%), neutropenia (33%), febrile neutropenia (28%), and thrombocytopenia (28%). Overall, the response rate with SEL-VEN was 21%. Two (11%) patients, one with prior allo-HSCT and one with prior VEN and both treated with SEL 80 mg/week, experienced complete remissions, with duration of response of 7 and 9.1 months, respectively. After a median follow up of 3.0 (range, 0.6-15.4) months, median event-free survival was 2.4 (95% CI: 1.9-12.1) months and median overall survival was 6.4 (95% CI: 2.5-12.1) months. In conclusion, SEL-VEN was feasible and active in a heavily pretreated AML cohort, with no new toxicity signal, but survival outcomes remained poor. The second-generation XPO1-inhibitor eltanexor, combined with VEN may further improve outcomes in VEN resistant AML in an ongoing study (NCT06399640).
    DOI:  https://doi.org/10.1002/ajh.70266
  8. Front Oncol. 2026 ;16 1735418
      TP53-mutated acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS) represent a biologically and clinically distinct subset of myeloid malignancies characterized by poor prognosis, resistance to standard therapies, and high rates of relapse. TP53 mutations, particularly biallelic are frequently associated with complex karyotypes and confer profound chemoresistance. Although hypomethylating agents and venetoclax-based combinations provide modest benefit, durable remissions remain rare. Novel therapeutic strategies targeting mutant p53, restoring wild-type function, or exploiting synthetic lethal pathways are under active investigation. This review aims to summarize current knowledge on the biology of TP53, prognostic implications, and therapeutic landscape of TP53-mutated AML/MDS, ongoing and past clinical trials in TP53-mutated AML/MDS patients, emphasizing the need for precision-guided, multimodal approaches to improve outcomes in this high-risk group.
    Keywords:  TP53 mutation; acute myeloid leukemia; allogeneic stem cell transplantation; hypomethylating agents; myelodysplastic syndromes; p53; venetoclax
    DOI:  https://doi.org/10.3389/fonc.2026.1735418
  9. Clin Lymphoma Myeloma Leuk. 2026 Feb 14. pii: S2152-2650(26)00039-X. [Epub ahead of print]
       INTRODUCTION: Improvements in splenomegaly, anemia, and overall survival (OS) are key considerations in the management of patients with myelofibrosis. In the phase III SIMPLIFY-1 trial (NCT01969838), momelotinib was noninferior to ruxolitinib for spleen volume reduction ≥ 35% (SVR35) and nominally superior for transfusion independence (TI) at week 24 in Janus kinase (JAK) inhibitor-naive patients. However, the relative impact of these endpoints on OS in anemic patients has not been described.
    MATERIALS AND METHODS: The present post hoc analysis evaluated week 24 SVR35, TI, and dual responses (SVR35 + TI) in patients with baseline hemoglobin < 10 g/dL.
    RESULTS: SVR35 rates were similar overall with momelotinib versus ruxolitinib (27/86 [31%] vs. 31/94 [33%]), but higher with momelotinib in the baseline platelets < 200 × 109/L subgroup (19/49 [39%] vs. 8/47 [17%]) and with ruxolitinib in the baseline platelets ≥ 200 × 109/L subgroup (8/37 [22%] vs. 23/47 [49%]). Week 24 SVR35 + TI was also more common with momelotinib (23/86 [27%]) than with ruxolitinib (7/94 [7%]). Subsequent OS analysis focused on the momelotinib arm only, as the crossover trial design precluded analysis of long-term OS with ruxolitinib. OS was longer in patients who were transfusion independent and/or achieved SVR35 at week 24 versus those who met neither endpoint (TI alone: n = 17, hazard ratio [HR], 0.25 [95% CI, 0.09-0.70]; SVR35 + TI: n = 23, HR, 0.40 [95% CI, 0.18-0.87]).
    CONCLUSION: These results highlight that both spleen- and anemia-related benefits of momelotinib correlate with improved OS, supporting prioritization of TI in anemic patients for optimal long-term outcomes, and suggest that momelotinib may be the preferred JAK inhibitor in anemic patients with platelets < 200 × 109/L.
    Keywords:  Hemoglobin; JAK inhibition; Platelet counts; Spleen response; Survival prediction
    DOI:  https://doi.org/10.1016/j.clml.2026.02.004
  10. Blood Neoplasia. 2026 May;3(2): 100202
      Targeted therapies, such as the BCL-2 inhibitor venetoclax, have expanded the treatment options for patients with acute myeloid leukemia (AML), but survival remains poor because of drug resistance and disease relapse. We found that the translation initiation factor EIF4A1, which unwinds complex messenger RNA structures in the 5' untranslated region (UTR) of oncogenic transcripts, is highly expressed in AML stem- and progenitor-like cells relative to healthy hematopoietic stem and progenitor cells. Inhibition of eukaryotic initiation factor 4A (eIF4A) with the first-in-class small molecule zotatifin reduces the translation efficiency of transcripts related to the cell cycle and oncogenic signaling via the PI3K/AKT/mTOR pathway, as shown by ribosome profiling and gene set enrichment analysis. Western blot analysis corroborated these findings and demonstrated the downregulation of AKT, STAT-5, and MCL-1, factors implicated in resistance to venetoclax-based regimens. The combination of zotatifin and venetoclax synergistically kills AML cells in vitro and induces apoptosis across AML genotypes with selectivity toward progenitor-like cells in primary AML bone marrow (BM); however, its effect in primary healthy BM is limited. Using 3 in vivo xenograft models derived from patients with relapsed/refractory AML, the combination significantly suppressed the tumor burden and prolonged survival. These results support eIF4A-mediated protein translation as a therapeutic target in AML and highlight the potential of zotatifin and venetoclax in relapsed/refractory disease.
    DOI:  https://doi.org/10.1016/j.bneo.2026.100202
  11. Exp Hematol. 2026 Mar 06. pii: S0301-472X(26)00036-6. [Epub ahead of print] 105403
      Overexpression of MYC is a common convergent consequence of genetic driver mutations in acute myeloid leukemia (AML). However, despite extensive research, the mechanisms by which this proto-oncogene promotes leukemogenesis remain incompletely understood. Here, we developed models of deregulated MYC expression in human pluripotent stem cell (hPSC)-derived myelopoiesis. We demonstrate that MYC overexpression from the endogenous locus, which maintains physiological regulation, results in a transient and reversible differentiation block that is insufficient for leukemogenesis. Instead, constitutive MYC overexpression from ectopic alleles, which results in a persistent block of differentiation, is necessary for driving and sustaining leukemia-associated phenotypes. These phenotypes result from the widespread disruption of epigenetic landscapes and transcription factor networks induced by MYC overexpression, underpinning differentiation arrest. Our findings shed new light on the mechanisms underlying MYC-induced malignant transformation and leukemogenesis, particularly the interplay between cellular differentiation and oncogenesis. TEASER ABSTRACT: Using hPSC-derived myelopoiesis models, we show that MYC overexpression from its endogenous locus causes a transient, reversible differentiation block that is insufficient for leukemogenesis, whereas constitutive ectopic MYC produces a persistent differentiation arrest that drives leukemia-associated phenotypes. Persistent MYC rewires epigenetic landscapes and transcription factor networks, revealing that sustained deregulation, not expression alone, underlies MYC-driven leukemic transformation.
    Keywords:  Acute Myeloid Leukemia (AML); MYC oncogene; cell differentiation; hPSC-derived myelopoiesis; leukemogenesis
    DOI:  https://doi.org/10.1016/j.exphem.2026.105403
  12. Blood Neoplasia. 2026 May;3(2): 100198
      Acute myeloid leukemia with myelodysplasia-related changes (AML-MRC) is a high-risk AML subtype defined by cytogenetic or genetic changes. We evaluated the relevance of AML-MRC classifications in a contemporary cohort of intensively treated patients with AML. We retrospectively reviewed 496 consecutive patients receiving induction chemotherapy for AML between 2016 and 2023 at a single center. Patients were categorized using both World Health Organization 2017 (MRC-2017) and International Consensus Classification 2022 (MRC-2022) criteria. Outcomes were compared using χ2 or Kaplan-Meier. MRC-2017 and MRC-2022 criteria identified 113 (22.8%) and 147 (29.6%) patients, respectively; 42.1% (77/183) met both definitions. Patients with AML classified by either definition had worse event-free survival (EFS) vs all patients with AML (MRC-2017: median, 1.4 vs 11.6 months; P < .001; MRC-2022: median, 1.9 vs 10.5 months; P = .019). Overall survival (OS) was inferior for MRC-2017 vs all patients with AML (median, 19.7 vs 53.1 months; P < .001) but not for MRC-2022. In the MRC-2022 mutation group, patients with a splice or transcription factor (TF) mutation had worse EFS vs mixed or chromatin modifier mutations (1.10 vs 5.35 vs 7.40 months, P = .002). Landmark analysis after allogeneic hematopoietic cell transplantation (allo-HCT) showed better 2-year OS for MRC-2022 vs non-MRC-2022 patients (77.0% vs 62.7%, P = .015). Multivariate analysis confirmed the EFS impact of TF/splice mutations (hazard ratio [HR], 1.65; 95% confidence interval [CI], 1.20-2.27; P = .002) and allo-HCT (HR, 0.49; 95% CI, 0.34-0.69; P < .001). These findings highlight the heterogeneity within the MRC-2022 group, particularly with mutation type and transplant status. Further refinement of AML-MRC risk stratification is needed.
    DOI:  https://doi.org/10.1016/j.bneo.2026.100198
  13. Biochem J. 2026 Mar 12. pii: BCJ20253463. [Epub ahead of print]
      The myeloid oncogene TRIB2 is a key driver of acute myeloid leukaemia (AML) pathogenesis, promoting chemoresistance and blocking differentiation through ubiquitin-mediated degradation of the C/EBPα transcription factor. Despite its stable and sometimes elevated expression across AML subtypes, TRIB2 remains a clinically-untargeted vulnerability. Here, we present a comprehensive investigation into TRIB2 degradation mechanisms using multimodal approaches, including CRISPR knockout, mutational protein stability, small molecule TRIB2 engagement and evaluation of a novel targeted protein degrader (TRIB2-PROTAC). We identify Afatinib, a multi-ERBB covalent inhibitor, as a rapid inducer of TRIB2 degradation, triggering AML cell death potentially via signalling pathways distinct from ERBB. Importantly, TRIB2 degradation synergized with cytarabine, the frontline AML chemotherapy, amplifying therapeutic efficacy. Mapping of TRIB2 ubiquitination sites revealed Lys-63 as critical for its own proteolytic turnover, and a Lys to Arg degradation-resistant mutant (KallR) conferred enhanced chemoresistance and increased leukaemic engraftment in vivo. CRISPR-mediated TRIB2 knockout validated an essential role in AML cell survival. Consistently, the novel TRIB2-PROTAC (compound 5K) achieved robust TRIB2 degradation and AML cell killing at low micromolar concentrations. These findings establish TRIB2 as a compelling therapeutic target in AML and demonstrate that leveraging the ubiquitin-proteasome system to degrade TRIB2 offers a promising strategy to overcome chemoresistance. This work provides strong preclinical rationale for the development of TRIB2-targeting therapies in AML.
    Keywords:  Acute myeloid leukaemia; Chemotherapy resistance; Pseudokinases; Tribbles; Ubiquitin proteasome system
    DOI:  https://doi.org/10.1042/BCJ20253463
  14. EJHaem. 2026 Apr;7(2): e70261
       Background: Older adults with acute myeloid leukemia (AML) have inferior outcomes, yet current genetic risk models do not explicitly account for how age modifies the prognostic impact of molecular features. We hypothesized that integrating apoptosis and p53-related gene expression with recurrent mutations would improve prediction of complete remission (CR) and 2-year overall survival (OS), particularly across age groups.
    Methods: Using the BeatAML2 dataset (805 patients; 942 specimens), we built two cohorts: a clinical cohort of 916 adults with full data and an expression-linked cohort of 852 with matched RNA-seq. Patients were divided into four age groups 18-30, 30-45, 45-60, and 60+ years. We tested whether adding expression of 12 apoptosis and p53-related genes to five well-known mutations, that is TP53, NPM1, FLT3, RUNX1, and ASXL1, could improve the prediction of CR and 2-year OS.
    Results: Adding gene expression improved predictive performance across models. For 2-year OS, AUCs rose from 0.765 to 0.772 in XGBoost, 0.703 to 0.843 in Random Forest, and 0.697 to 0.721 in Logistic Regression. For CR, performance improved from 0.770 to 0.851 in XGBoost, 0.811 to 0.861 in Random Forest, and 0.731 to 0.696 in Logistic Regression. Calibration was strongest for tree-based models, and reclassification improved most with XGBoost. Multivariable regression confirmed TP53 as the most adverse marker for OS (HR: 3.07), with added risk from ASXL1 (HR: 1.53) and FLT3 (HR: 1.39). NPM1 increased the chance of remission (OR: 2.47) but did not extend survival. SHAP analysis showed that age remained the leading predictor of OS. Among genes, CHEK2 expression was most important for survival, especially in patients 60 years and older, while CCNG1 expression best predicted remission, along with BAX and MCL1.
    Conclusions: These results demonstrate that combining gene expression with recurrent mutations makes risk prediction more accurate, especially in older patients who formed the largest group and had the poorest outcomes. Although treatment variables were not included and analysis focused on selected genes, these findings support incorporation of expression-based features into genetic risk models and warrant prospective validation.
    Trial Registration: The authors have confirmed clinical trial registration is not needed for this submission.
    DOI:  https://doi.org/10.1002/jha2.70261
  15. Cell Death Dis. 2026 Mar 07.
      Histone deacetylase 6 (HDAC6) has emerged as a promising therapeutic target in cancer due to its immunomodulatory effects. While its prognostic significance remains debated, we demonstrate that HDAC6 loss significantly impairs myeloid leukemia progression in vivo, despite having no functional impact on leukemia cell proliferation in vitro. Global proteome and secretome profiling of HDAC6-knockout (KO) cells revealed upregulation of several immune-related modulators, including RNase T2, a tumor suppressor known to modulate the tumor microenvironment. Notably, RNase T2 upregulation upon HDAC6 loss was observed in myeloid leukemia cells but not in lymphoblastic leukemia cells. Moreover, pharmacological inhibition of HDAC6 recapitulated this phenotype, leading to RNase T2 upregulation in myeloid leukemia cells. ATAC-seq revealed increased chromatin accessibility of RNase T2 following HDAC6 loss, highlighting a functionally epigenetic regulatory contribution. Further functional assays conducted in an immunocompetent setting, both ex vivo and in vivo, demonstrated that HDAC6 inhibition sensitized murine myeloid leukemia cells to broad CD8+ T cell activation as evidenced by increased TNFα and CD107a expression. Consistently, in a syngeneic murine model, HDAC6 inhibition restricted the growth of myeloid leukemia cells. Moreover, an extended drug screening analysis identified Cytarabine and Clofarabine as significantly synergizing with HDAC6 inhibitor (Ricolinostat) in myeloid leukemia cell lines and in patient-derived xenograft (PDX) cells, while showing limited synergy in lymphoid leukemia cell lines, PDX, or healthy control cells. These findings suggest that HDAC6 represents a promising therapeutic target in myeloid lineage-derived leukemia cells by simultaneously enhancing immune activation and increasing chemosensitivity.
    DOI:  https://doi.org/10.1038/s41419-026-08541-3
  16. Haematologica. 2026 Mar 12.
      NOP2/Sun RNA methyltransferase family member 2 (NSUN2) catalyzes 5-methylcytosine (m5C) modifications on RNA to regulate mRNA stability. However, its roles in normal hematopoiesis and leukemogenesis remain poorly understood. Here, we show that NSUN2 is markedly upregulated in primary AML patient samples compared with normal hematopoietic cells. NSUN2 knockdown (KD) impaired AML cell proliferation, induced apoptosis, and reduced colony formation. Genetic ablation of Nsun2 in an MLL-AF9 (MA9)-transformed murine AML model substantially impaired leukemia stem cell (LSC) self-renewal and prolonged overall survival (OS), while sparing normal hematopoiesis, highlighting NSUN2 as a potential therapeutic target. Notably, wild-type NSUN2, but not catalytically inactive mutants, restored LSC function and leukemogenesis in NSUN2-deficient AML cells, indicating that these effects are mô€€€C-dependent. Mechanistically, NSUN2 stabilized FosB proto-oncogene (FOSB) mRNA via mô€€€C modification at nucleotide 3656 in the 3′-UTR, thereby upregulating FOSB expression. In turn, FOSB transcriptionally activated NSUN2, forming a feedforward regulatory loop. Furthermore, FOSB promoted expression of the anti-apoptotic regulator B-cell lymphoma-2-like protein 1 (BCL2L1) by directly binding to its promoter. In conclusion, these findings uncover a novel NSUN2-FOSB-BCL2L1 axis that drives AML leukemogenesis in an m5C-dependent manner, suggesting the therapeutic potential for targeting this pathway.
    DOI:  https://doi.org/10.3324/haematol.2025.289250
  17. Blood Neoplasia. 2026 May;3(2): 100190
      Acute myeloid leukemia (AML) is caused by uncontrolled proliferation and impaired differentiation of hematopoietic stem and progenitor cells. Historically, research has emphasized the role of protein-coding genes in the development of AML. However, with the human genome project revealing that 98% of the transcriptome consists of non-protein-coding RNAs, recent studies have explored how the large classes of noncoding RNAs (ncRNAs) contribute to AML. Although there are many types of ncRNAs, much attention has been placed on understanding the function of long ncRNAs (lncRNAs) and small ncRNAs known as microRNAs (miRNAs). lncRNAs are >200 nucleotides, whereas mature miRNAs are typically 18 to 25 nucleotides. lncRNAs are involved in miRNA and protein sequestration and act as transcriptional and translational regulators, whereas miRNAs facilitate mRNA degradation and translational inhibition. In addition to lncRNAs and miRNAs, two additional types of ncRNAs, namely small nucleolar RNAs (snoRNAs) and circular RNAs (circRNAs), have recently garnered attention for their roles in AML. Here, we discuss how these four distinct classes of ncRNAs may aid in disease diagnosis and prognosis as well as the mechanisms by which their dysregulation contributes to AML.
    DOI:  https://doi.org/10.1016/j.bneo.2026.100190
  18. J Clin Pathol. 2026 Mar 12. pii: jcp-2025-210460. [Epub ahead of print]
       AIMS: Although acute myeloid leukaemia (AML), myelodysplasia-related (AML-MR), can be defined solely by molecular abnormalities, legacy studies did not analyse the KMT2A gene. The KMT2A-partial tandem duplication (KMT2A-PTD) is described in myelodysplastic neoplasms (MDS) as well as AML. We describe the clinical, morphological, immunophenotypic and molecular features of AML harbouring KMT2A-PTD.
    METHODS: We studied 802 adult AML patients, for whom next-generation sequencing- based mutation and copy number analysis were available. For the patients harbouring KMT2A-PTD, the immunophenotypic analysis including measurable residual disease (MRD), mutational landscape and the patient outcomes were analysed.
    RESULTS: We identified 45 patients of de novo and secondary AML harbouring KMT2A-PTD. Morphological dysplasia and immunophenotypic abnormalities, described in MDS, were observed in 35.6% and 40% of de novo cases respectively. Furthermore, 44% of AML with KMT2A-PTD could be diagnosed as AML-MR based on cytogenetics or genomics. We observed progenitor abnormalities, commonly aberrant CD7 (33%) and CD15 (59%), and granulocytic abnormalities like loss of side scatter (50%), asynchronous maturation patterns and loss of CD177 in mature granulocytes. Frequent mutations involving IDH2 (30% of which were R172), FLT3 (32% each), RUNX1 (29%), DNMT3A and U2AF1 (24% each) were noted. We also found that more than 25% of patients did not achieve morphological remission, and the remaining 60% were MRD positive. The outcomes of AML with KMT2A-PTD with or without MR-associated abnormalities were similar.
    CONCLUSIONS: AML harbouring KMT2A-PTD frequently displays MR immunophenotypic abnormalities and is frequently associated with AML-MR type mutations. In addition, they have similar outcomes as compared with AML-MR.
    Keywords:  IMMUNOPHENOTYPING; Leukemia, Myeloid; MOLECULAR BIOLOGY; MYELODYSPLASIA
    DOI:  https://doi.org/10.1136/jcp-2025-210460
  19. Nature. 2026 Mar 11.
      Immune responses to parasite infection involve the increased production of basophils and eosinophils. These two myeloid cell types have key roles in type 2 anti-parasite immunity1 and rely on GATA family transcription factors for their specification2,3. The first committed step in basophil and eosinophil production is generation of basophil-eosinophil-mast cell progenitors (BEMPs) from oligopotent erythroid-primed multipotent progenitors (EMPPs). However, it is not well established how immune responses act on progenitors to initiate type 2 myelopoiesis. Here we show that infection with the helminth Heligmosomoides polygyrus increases EMPP commitment to myeloid fate at the expense of erythropoiesis. Upon infection with H. polygyrus, the IL-33 alarmin accumulated in the bone marrow, causing EMPPs to upregulate the GATA co-factor LMO4 and preferentially differentiate into myeloid cells. LMO4 was sufficient to instruct myeloid fate in EMPPs by interacting with GATA2, displacing the FOG1 co-factor and redistributing GATA binding from megakaryocyte-erythroid-specific to basophil, eosinophil and mast cell (BEM)-specific chromatin. Accordingly, mice carrying a GATA2 mutation that selectively impairs the LMO4-GATA2 interaction were deficient in GATA factor allocation to BEM chromatin, myeloid lineage commitment, basophil and eosinophil production, and parasite control. This identifies LMO4 as an IL-33-regulated master regulator of type 2 myelopoiesis, and transcription factor reallocation as a mechanism of lineage commitment.
    DOI:  https://doi.org/10.1038/s41586-026-10256-6
  20. Blood Cancer J. 2026 Mar 12.
      Cytogenetic abnormalities and recurrent gene mutations are central to the biology and prognosis of myelodysplastic syndromes/neoplasms (MDS); however, the significance of patients without detectable genomic lesions remains unclear. In this retrospective analysis of 758 patients with primary MDS, the participants were stratified according to the presence of gene mutations and/or cytogenetic abnormalities. Approximately twenty percent lacked both abnormalities and were characterized by younger age, female predominance, lower IPSS-R/IPSS-M risk, and the most favorable survival outcomes. Bulk RNA sequencing further revealed that mutation-negative/normal-karyotype MDS was characterized by immune-metabolic transcriptional programs, whereas genomically altered disease exhibited inflammatory, proliferative, and stress-response signatures, indicating a biologically distinct low-risk state. Therefore, patients without genomic abnormalities appear to constitute a biologically distinct subgroup of MDS. To refine risk assessment beyond IPSS-M systems, a multivariable Cox-based nomogram was developed incorporating age, ferritin level >800 ng/mL, lactate dehydrogenase level >200 U/L, bone marrow fibrosis, and IPSS-M. The proposed model outperformed IPSS-M alone and remained robust following bootstrap validation, allowing individualized 12- and 36-month survival estimation. These findings underscore the limitations of relying solely on molecular assays for risk stratification and support the integration of clinical, genomic, and model-based predictors to improve classification and guide individualized MDS management.
    DOI:  https://doi.org/10.1038/s41408-026-01456-4
  21. Hemasphere. 2026 Mar;10(3): e70334
      Hematopoietic stem cells (HSCs) are responsible for replenishing blood cells under stress conditions through increasing proliferation and differentiation. After the hematopoietic function has reconstructed, HSCs must re-enter a quiescent state to avoid their depletion, whereas the underlying mechanisms remain to be elucidated. Here, we show that the translocation of nuclear receptor coactivator 2 (NCOA2) into the nucleus is gradually increased in HSCs during the hematopoietic recovery phase after sub-lethal dose irradiation (IR). Although deletion of NCOA2 only slightly affects the steady state hematopoiesis, its deficiency leads to HSC pool exhaustion and delayed hematopoietic recovery after exposure to IR. Further investigations reveal that loss of NCOA2 decreases the quiescence, survival, and long-term reconstituting ability of HSCs following IR due to increased mitochondria-derived oxidative stress. Mechanistically, NCOA2 promotes the clearance of activated or damaged mitochondria by coactivating FOXO3a-dependent transcription of PINK1, which drives HSCs to return to quiescence after being activated by IR stress. Collectively, our findings demonstrate a critical role of NCOA2 in facilitating the restoration of HSC homeostasis after IR via the FOXO3a-PINK1-mediated mitophagy axis and thus provide an additional strategy to prevent hematopoietic failure induced by IR.
    DOI:  https://doi.org/10.1002/hem3.70334
  22. Blood Rev. 2026 Mar 06. pii: S0268-960X(26)00024-X. [Epub ahead of print] 101385
      Ph-chromosome-negative myelo-proliferative neoplasms (MPNs) are characterized by over-production of mature blood cells driven by acquired somatic variants in JAK2, CALR and MPL in most instances. Ph-chromosome-negative MPNs are genetically and clinically heterogeneous. These variants are detected in blood cells of > 3 per cent of normal people but few develop Ph-chromosome-negative MPNs. People with Ph-chromosome-negative MPNs often have sub-clones which are important for disease evolution. Clonal architecture is shaped by hereditary factors, inflammation and environmental exposures such as some chemicals and cytotoxic drugs. Clonal architecture correlates with phenotype, prognosis and risk of leukaemia transformation. Recent advances like single-cell DNA sequencing (scDNA-seq) provide data on the clonal dynamics of Ph-chromosome-negative MPNs. We comprehensively review genomic profiles, clonal evolution and risk of leukaemia transformation, emphasizing the importance of clonal architecture.
    Keywords:  Acute myeloid leukaemia; CALR; Clonal evolution; JAK2; MPL; MPNs; Myelo-proliferative neoplasms
    DOI:  https://doi.org/10.1016/j.blre.2026.101385
  23. Br J Haematol. 2026 Mar 14.
      A truncated derivative of Fibroblast growth factor receptor-1 (FGFR1) (tnFGFR1) independently transforms haematopoietic stem cells leading to a stem cell-like leukaemia/lymphoma syndrome (SCLL). In mouse models, these transformed cells show extensive genetic reprogramming that is distinct from cells transformed with full-length chimeric FGFR1 kinases. We now show that the truncated derivative is insensitive to kinase inhibitors, and its increased expression is related to resistance to kinase inhibitors. Chromatin immunoprecipitation (ChIP) analysis shows that tnFGFR1 can occupy the promoters of the Myc, Flt3 and Kit genes suggesting a transcription regulatory function. However, without a deoxyribonucleic acid-binding motif, tnFGFR1 must interact with binding partners that supply this function. To define this potential regulatory complex, we performed immunoprecipitation-mass spectroscopy (IP-MS) and demonstrate that tnFGFR1 is present in a complex with the splicing factor proline- and glutamine-rich (SFPQ) protein and non-POU domain-containing octamer-binding protein (NONO) protein. In addition, this protein complex is present on the promoters of target genes Flt3 and Kit, and knockdown of either SFPQ or NONO prevents activation of their target genes. In addition, treatment with the NONO inhibitor auranofin suppresses cell proliferation of tnFGFR1-transformed cells in vitro mitigating leukaemia progression in vivo in a mouse model. Thus, future targeting of this tnFGFR1 transcription complex may provide a means for treating tnFGFR1-driven leukaemia.
    Keywords:  FGFR1; NONO; SFPQ; leukaemia; resistance
    DOI:  https://doi.org/10.1111/bjh.70426
  24. Blood. 2026 Mar 10. pii: blood.2025032216. [Epub ahead of print]
      Transposable elements (TEs) are emerging regulators of hematopoiesis and leukemia, creating vulnerabilities exploitable for therapy. Recent evidence shows that TE reactivation induces innate immune signalling, DNA damage responses and dependence on Poly(ADP-ribose) polymerase (PARP)-mediated protection, enabling synthetic lethality with PARP inhibition even in homologous-recombination-proficient leukemias with epigenetic gene mutations. In this article, we highlight the biology underpinning this novel TE-PARP axis, its therapeutic implications and strategies to expand beyond HR-deficient cancers through rational combinations with immunotherapy and refined patient stratification.
    DOI:  https://doi.org/10.1182/blood.2025032216
  25. Blood Neoplasia. 2026 May;3(2): 100193
      Mutations in calreticulin (CALR) represent the second most common oncogenic driver of myeloproliferative neoplasms. CALR mutations result in the introduction of a neomorphic C-terminal tail that is absent from the normal proteome, which has positioned it as a compelling target for immunotherapeutic intervention. Early studies have demonstrated mutant CALR can elicit T-cell responses, laying the foundation for efforts to exploit this vulnerability through vaccines, antibody-based strategies, and T-cell-based therapies. Here, we summarize the current understanding of normal and mutant CALR biology, discuss progress in developing CALR-directed immunotherapies, and highlight the challenges and opportunities for translating these approaches into the clinic.
    DOI:  https://doi.org/10.1016/j.bneo.2026.100193
  26. Haematologica. 2026 Mar 12.
      Targeted therapies have made near normal life span an attainable goal for many patients with chronic phase (CP) chronic myeloid leukemia (CML). Most patients require years of therapy and not everyone may be able to discontinue treatment permanently without CML recurrence. BCR::ABL1 targeted tyrosine kinase inhibitors (TKIs) including ATP binding site and allosteric inhibitors that bind to the myristoyl pocket are associated with treatment-emergent adverse events (TEAEs) that may compromise quality of life and well-being. Although alternative treatment options exist, side effects may persist, or new ones occur after a therapy switch. Using a case-based approach, this review examines the incidence of non-hematologic and hematologic TEAEs with specific therapies, provides guidance on AE management, and describes the impact of therapy dose reduction on efficacy and tolerability.
    DOI:  https://doi.org/10.3324/haematol.2025.288334
  27. iScience. 2026 Mar 20. 29(3): 114921
      Hematopoietic stem and progenitor cells (HSPCs), including multipotent progenitors (MPPs), sustain lifelong blood production by integrating intrinsic metabolic and epigenetic mechanisms with extrinsic cues from the bone marrow (BM) niche. Epigenetic mechanisms interact with metabolic pathways to establish a coordinated network that supports HSPC maintenance and differentiation. Spatially defined signals within BM niches shape HSPC metabolic and epigenetic states and govern lineage specification. Herein, we review recent advances on the bidirectional relationship between metabolism and epigenetics in HSPCs, emphasizing how both intrinsic and niche-derived factors regulate fate decisions under steady-state and pathological conditions. Particular attention is given to the CXCL12/CXCR4 signaling axis, a central regulator of HSPC retention, migration, and quiescence, and its emerging role in orchestrating metabolic and epigenetic mechanisms. Integrating intrinsic regulatory networks with dynamic extrinsic signals provide a conceptual framework for understanding HSPC fate and may uncover strategies for regenerative medicine and hematological disease therapy.
    Keywords:  biological sciences; cell biology; molecular biology
    DOI:  https://doi.org/10.1016/j.isci.2026.114921
  28. Expert Opin Pharmacother. 2026 Mar 10.
       INTRODUCTION: Myelodysplastic syndromes/neoplasms (MDS) comprise a heterogeneous group of clonal myeloid malignancies characterized by ineffective hematopoiesis and a variably increased risk of progression to acute myeloid leukemia (AML). Higher-risk MDS, as defined by the revised- and molecular International Prognostic Scoring System, remains largely incurable except through allogeneic hematopoietic stem cell transplantation. Management continues to rely primarily on hypomethylating agents, which were approved nearly two decades ago and remain the cornerstone of therapy despite their modest efficacy.
    AREAS COVERED: Advances in molecular profiling and next-generation sequencing have greatly improved disease classification and prognostication tools. However, with the exception of IDH1 inhibitor ivosidenib, targeted therapies in higher-risk MDS remain the exception to the rule. Although numerous early-phase clinical trials have yielded promising signals, phase III trials have repeatedly failed to replicate these findings. This largely reflects the intrinsic biological complexity and heterogeneity of MDS, as well as logistical challenges in trial design and execution.
    EXPERT OPINION: Examining the history and evolution of investigational pharmacotherapy in higher-risk MDS may help identify where efforts to translate early phase successes into successful phase III trials have faltered. The expanding biological knowledge base and the design of better therapies will hopefully pave the way for future therapeutic breakthroughs.
    Keywords:  Higher-risk MDS; cell therapy; clinical trials; targeted therapy
    DOI:  https://doi.org/10.1080/14656566.2026.2644384
  29. Nucleic Acids Res. 2026 Feb 24. pii: gkag219. [Epub ahead of print]54(5):
      DDX41 mutations are associated with myeloid neoplasms, including myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML), and missense mutant R525H is seen in 67% of patients; however, its molecular pathogenesis is unknown. Using DDX41 knockout (KO) cells, we found that these cells were sensitive to bleomycin, camptothecin, and UV. DDX41 deficiency led to increased genomic instability, indicated by elevated DNA double-strand breaks (DSBs) and comet tails. We found that R-loop formation increased in DDX41-KO cells. DDX41 wild-type (WT) protein resolved DNA:RNA hybrid of R-loops in vitro, but the mutant R525H failed. DDX41-R525H expressing cells were sensitive to DNA damage agents and had significantly more R-loops than DDX41-WT expressing cells. Interestingly, DDX41 colocalized with DSB marker γH2AX and R-loop marker S9.6, and knockdown of DDX41 in the U2OS GFP reporter cells resulted in reduced homologous recombination (HR) repair. Moreover, increased and prolonged RPA and reduced RAD51 foci were found in DDX41 KO and DDX41-R525H expressing cells, indicating a defect in the transition from end resection to RAD51 filament assembly. Overall, our results suggest that DDX41 utilizes its unwinding activity to resolve R-loops, which may play a key role in HR-based repair, and dysregulation of this pathway may lead to MDS/AML.
    DOI:  https://doi.org/10.1093/nar/gkag219
  30. NPJ Precis Oncol. 2026 Mar 11.
      FLT3-mutated acute myeloid leukemia (AML) remains difficult to treat due to frequent resistance to FLT3 inhibitors like midostaurin. In this study, we observed a progenitor-like CD38+CD45RA+ leukemic cell population that may be associated with midostaurin resistance. Midostaurin-resistant cells display disrupted membrane architecture and a shift in signaling from STAT5 to PI3K/AKT, favoring survival over apoptosis. Functional drug testing was consistent with clinical response to midostaurin, and together with multi-omic profiling, including single-cell and proteomic analyses, indicated the presence and relevance of this resistant phenotype. Drug combination screening revealed that co-targeting with SMAC mimetics restores apoptotic competence and selectively depletes the resistant population when combined with midostaurin. In contrast, venetoclax combinations preferentially affected CD34hi cells, underscoring distinct subpopulation vulnerabilities. These findings may point to a biologically relevant mechanism underlying midostaurin resistance.
    DOI:  https://doi.org/10.1038/s41698-026-01363-8