bims-tremyl Biomed News
on Therapy resistance biology in myeloid leukemia
Issue of 2025–06–22
thirty-one papers selected by
Paolo Gallipoli, Barts Cancer Institute, Queen Mary University of London



  1. Cancer Discov. 2025 Jun 19.
      Targeting the MYST acetyltransferases are an exciting therapeutic opportunity in acute myeloid leukaemia (AML). Here we define the individual and combined contribution of KAT6A, KAT6B and KAT7, in range of AML models showing that although KAT6A/B inhibition is efficacious in some pre-clinical models, simultaneous targeting of KAT7, with the novel inhibitor PF-9363, markedly increases efficacy. KAT7 interacts with Menin and the MLL complex and is co-localised at chromatin to co-regulate oncogenic transcriptional programs. Focusing on MLL fusion oncoprotein (MLL-FP) AML, we show that inhibition of KAT6/KAT7 provides an orthogonal route to targeting Menin to disable the transcriptional activity of the MLL-FP. Combined inhibition rapidly evicts the MLL-FP from chromatin, potently represses oncogenic transcription and overcomes primary resistance to Menin inhibitors. Notably, KAT7 remains an important targetable dependency in acquired genetic/non-genetic resistance to Menin inhibition providing the molecular rationale for rapid clinical translation of combination therapy, particularly in MLL-FP AML.
    DOI:  https://doi.org/10.1158/2159-8290.CD-24-1517
  2. Blood. 2025 Jun 20. pii: blood.2024026417. [Epub ahead of print]
      Mutations in TP53 are mutually exclusive with other known drivers of myeloid transformation and define a distinct molecular subtype within de novo Acute Myeloid Leukemia (AML) that is associated with a complex karyotype, resistance to chemotherapy, and poor prognosis. Although TP53 defects are rare in de novo AML, biallelic mutations are a defining molecular feature of erythroleukemia. The genetic alterations that cooperate with defective TP53 to transform erythroid progenitors remain unknown. We found that loss of BAP1 (BRCA1 Associated Protein-1) co-occurs in one-third of patients with TP53-mutated AML, is associated with an erythroid-primed gene expression signature, and confers an additional adverse effect on overall survival. BAP1 is a tumor suppressor involved in the DNA damage response as well as epigenetic regulation through histone H2AK119 de-ubiquitination. While Bap1KO mice develop myelodysplasia with prominent dyserythropoiesis, combined deletion of Bap1 and Trp53 caused transplantable erythroleukemia, and occasionally mixed AML, mirroring the heterogeneity of human disease. Bulk and single-cell RNA-seq coupled to ChIP-seq in hematopoietic progenitors revealed that Bap1 loss triggers a proinflammatory response and cooperates with Trp53 deficiency to transform erythroid-primed multipotent progenitors. Mechanistically, genomic instability led to the development of erythroleukemia, while epigenetic deregulation caused myelomonocytic skewing suggesting a dichotomous and context dependent role for BAP1. We also demonstrate that BAP1 deficient erythroleukemia is dependent on BCL2L1 expression and is sensitive to BCL-xL inhibitors in vivo.
    DOI:  https://doi.org/10.1182/blood.2024026417
  3. Cancer Discov. 2025 Jun 19.
      NUP98 fusion oncoproteins (FOs) are a hallmark of childhood acute myeloid leukemia (AML). NUP98 FOs drive leukemogenesis through phase-separated condensate formation and maintenance of an active chromatin landscape at stem cell-associated genes in cooperation with epigenetic regulators. Here we show that MYST family histone acetyltransferase (HAT) complex proteins including KAT6A/MOZ, KAT7/HBO1, and the common KAT6A/7 complex subunit BRPF1 associate with NUP98 FOs on chromatin and within condensates. MYST HATs are molecular dependencies in NUP98-rearranged (NUP98-r) leukemia, and genetic inactivation or pharmacologic inhibition of Kat6a and Kat7 impairs NUP98-r cell fitness. KAT6A/7 inhibition decreased global H3K23ac levels, displaced NUP98::HOXA9 from chromatin at the Meis1 locus, and led to myeloid cell differentiation. Additionally, KAT6A/7 inhibition decreased leukemic burden in multiple NUP98-r leukemia xenograft mouse models, synergized with Menin inhibitor treatment, and was efficacious in Menin inhibitor-resistant cells. In summary, we show that MYST family HATs are therapeutically actionable dependencies in NUP98-r AML.
    DOI:  https://doi.org/10.1158/2159-8290.CD-24-1772
  4. Hematol Oncol. 2025 Jun;43 Suppl 2 e70075
      The expansion of hematopoietic cell clones, carrying alterations in genes frequently mutated in hematologic malignancies, in the absence of altered hematopoietic cell counts or otherwise defined disease criteria, is termed clonal hematopoiesis (CH). CH is frequently detectable in aged individuals and associates with numerous detrimental health impacts. These impacts are highly dependent on the type of mutations and the cellular context in which they manifest. Mutations in the hematopoietic stem and progenitor cell (HSPC) compartment as well as in self-renewing more mature cells associate with increased risks of malignant disease, while mutations penetrating via hematopoiesis in non-self-renewing, mature cells associate with altered immune functions and consequent systemic effects, which can initiate or aggravate multiple non-malignant diseases. Here we review the definitions of CH, major genetic drivers and lineage penetrance, and we highlight how CH impacts on hematological and non-hematological conditions.
    Keywords:  clonal hematopoiesis; health outcomes; leukemia
    DOI:  https://doi.org/10.1002/hon.70075
  5. Leukemia. 2025 Jun 18.
    French Intergroup of Myeloproliferative Neoplasms (FIM)
      Genetic analyses have been included in scoring systems to improve the prognostic stratification of hematologic malignancies. Until now, molecular risk scores have not been included into the practical management of patients with polycythemia vera (PV). In this work, we studied 439 PV patients recruited from 15 French centers and described their mutational landscape using high-throughput sequencing. We detected an additional mutation in 53.3% of patients, 22.7% of them having 2 or more mutations. A Bayesian approach identified preferential associations between mutations. Based on these associations, we identified high molecular risk abnormalities in PV (PV-HMR), consisting in mutations in SRSF2, IDH1/2, EZH2 or NFE2 genes, copy number variations (CNV) and carrying 2 or more non-driver mutations. These PV-HMR were associated with decreased overall survival (OS) and/or transformation-free survival (TFS). Notably, ASXL1 mutations were not associated with a pejorative impact on OS or TFS when isolated. Based on these results, we developed a genomic 3-tier classification that efficiently predicted OS and more importantly TFS independently of age, sex, history of thrombosis and leukocyte and platelet counts. This model outperformed the IWG-PV and MIPSS-PV scoring systems in predicting the hematologic evolution of PV patients, which was confirmed in 2 external cohorts.
    DOI:  https://doi.org/10.1038/s41375-025-02660-0
  6. Br J Haematol. 2025 Jun 16.
      DNA methyltransferase inhibitors (DNMTis) are commonly used in treating chronic myelomonocytic leukaemia (CMML); however, data from prospective studies of DNMTis in CMML are limited. The present analysis evaluated the efficacy, safety and pharmacodynamics of the oral DNMTi decitabine/cedazuridine in the subset of patients with CMML from the phase 2 and 3 trials, which led to the approval of this agent for myelodysplastic syndromes and CMML in the United States and Canada. Potential prognostic factors also were analysed. In all, 34 patients with CMML were screened and 33 were treated. Most patients (76% [n = 25]) had myelodysplastic type-CMML and 77% (n = 24/31 with DNA available for sequencing) had intermediate-2 or high-risk disease noted by CMML-specific prognostic scoring systems. The overall response rate was 76%, with 21% (n = 7) of patients achieving a complete response. Nearly half of the 11 patients who were red blood cell-transfusion dependent at baseline (46%) attained transfusion independence for ≥12 weeks, which was associated with survival. Median overall and transformation-free survival were 35.7 and 28.3 months, respectively, and the safety profile was similar to that previously reported for decitabine. This analysis described the use of decitabine/cedazuridine in CMML from consecutive, prospective, randomised trials and illustrated a median survival of nearly 3 years.
    Keywords:  DNA methyltransferase inhibitors; chronic myelomonocytic leukaemia; decitabine/cedazuridine; hypomethylating agents; prognostic factors
    DOI:  https://doi.org/10.1111/bjh.20203
  7. Haematologica. 2025 Jun 19.
      Signal transducer and activator of transcription 3 (STAT3) is a well-described transcription factor that mediates oxidative phosphorylation and glutamine uptake in bulk acute myeloid leukemia (AML) cells and leukemic stem cells (LSCs). STAT3 has also been shown to translocate to the mitochondria in AML cells, and phosphorylation at the serine 727 (pSTAT3 S727) residue has been shown to be especially important for STAT3's mitochondrial functions. We demonstrate that inhibition of STAT3 results in impaired mitochondrial function and decreased leukemia cell viability. We discovered a novel interaction of STAT3 with voltage-dependent anion channel 1 (VDAC1) in the mitochondria which provides a mechanism through which STAT3 modulates mitochondrial function and cell survival. Through VDAC1, STAT3 regulates calcium and oxidative phosphorylation in the mitochondria. STAT3 and VDAC1 inhibition also result in significantly reduced engraftment potential of LSCs, including primary samples resistant to venetoclax. These results implicate STAT3 as a therapeutic target in AML.
    DOI:  https://doi.org/10.3324/haematol.2025.287352
  8. Blood Adv. 2025 Jun 15. pii: bloodadvances.2025016236. [Epub ahead of print]
      Limited data exist comparing haploidentical and mismatched unrelated donor (MMUD) hematopoietic cell transplantation (HCT) with post-transplantation cyclophosphamide (PTCy) for graft-versus-host disease prophylaxis, especially considering donor age. Herein, we report the outcomes of 660 haploidentical and 195 MMUD HCT recipients treated at MD Anderson Cancer Center. Beyond standard Cox Proportional Hazards modeling, we employed inverse probability of treatment weighting (IPTW), matched-pair analysis, and performed additional analysis by incorporating an external MMUD validation cohort from the Center for International Blood and Marrow Transplant Research (CIBMTR). The primary outcome was overall survival (OS). In multivariable analysis, haploidentical donors had a hazard ratio of 1.20, with a 95% confidence interval (CI), 0.93-1.54, p=.16 compared to the MMUD group. Donor age showed a non-linear association with OS. These findings were corroborated by IPTW, matched-pair analyses, and CIBMTR validation analyses. Exploratory analysis revealed inferior OS for older (>50 years) haploidentical donor group compared to younger (<30 years) MMUD recipients (HR 1.91, 95% CI 1.21-3.01, p=.005). Our analyses suggest that while donor type may play a role, there was a more prominent role for donor age in influencing OS. Moreover, our findings indicate a potential nuance wherein the impact of donor type may vary by donor age. Further research, particularly with larger cohorts, is needed to fully elucidate the complex and potentially interacting roles of donor type and donor age, along with HLA factors.
    DOI:  https://doi.org/10.1182/bloodadvances.2025016236
  9. Arch Pathol Lab Med. 2025 Jun 16.
       CONTEXT.—: Following the validation of a multicolor flow cytometry (MFC) assay for measurable residual disease (MRD) in acute myeloid leukemia (AML), this study examines its clinical applicability.
    OBJECTIVE.—: To evaluate the practicality and performance of MFC-based MRD detection in AML.
    DESIGN.—: Prospectively assessed AML MRD MFC in unselected AML patients achieving morphologic remission with follow-up studies, molecular genetics, and survival data.
    RESULTS.—: Among 379 patient bone marrow samples in this cohort, an interpretable result was obtained in 359 (95%). A total of 57 of the 359 cases (16%) were positive for MRD, and the most frequently observed immunophenotype was CD34+CD117+ myeloid (n = 46; 81%), followed by CD34-/CD117+ myeloid (n = 8; 14%) and monocytic (n = 3; 5%). Of 57 MRD+ cases, 6 (11%) had no leukemia-associated immunophenotypes available, and 16 of 51 (31%) with leukemia-associated immunophenotype for comparison exhibited significant immunophenotypic drift/switch, highlighting the importance of the "deviation from normal" approach. The remaining 302 cases were MRD negative; among these, 21 (6%) displayed a preleukemic immunophenotype that was associated with persistent clonal hematopoiesis in 18 patients (86%). A positive MFC result was strongly associated with subsequent follow-up positive MRD (41 of 45 [91%] versus 14 of 240 [6%], P < .01), morphologic relapse (42 of 55 [76%] versus 48 of 301 [16%], P < .01), an inferior overall survival (12.5 months versus not reached, P < .01), and leukemia-free survival (6.5 months versus not reached, P < .01). Among MRD-negative patients, a preleukemic phenotype was associated with a shorter overall survival (P = .03), but not leukemia-free survival (P = .16).
    CONCLUSIONS.—: Our study provides data-driven technical insights for laboratories considering MFC AML MRD implementation and offers strong evidence supporting the utility of MRD assessment by MFC in patients with AML undergoing various stages of treatment and surveillance.
    DOI:  https://doi.org/10.5858/arpa.2025-0053-OA
  10. Cell. 2025 Jun 12. pii: S0092-8674(25)00561-6. [Epub ahead of print]
      Developmental gene expression is regulated by the dynamic interplay of histone H3 lysine 4 (H3K4) and histone H3 lysine 27 (H3K27) methylation, yet the physiological roles of these epigenetic modifications remain incompletely understood. Here, we show that mice depleted for all forms of H3K4 methylation, using a dominant histone H3-lysine-4-to-methionine (H3K4M) mutation, succumb to a severe loss of all major blood cell types. H3K4M-expressing hematopoietic stem cells (HSCs) and committed progenitors are present at normal numbers, indicating that H3K4 methylation is dispensable for HSC maintenance and commitment but essential for progenitor cell maturation. Mechanistically, we reveal that H3K4 methylation opposes the deposition of repressive H3K27 methylation at differentiation-associated genes enriched for a bivalent (i.e., H3K4/H3K27-methylated) chromatin state in HSCs and progenitors. Indeed, by concomitantly suppressing H3K27 methylation in H3K4-methylation-depleted mice, we rescue the acute lethality, hematopoietic failure, and gene dysregulation. Our results provide functional evidence for the interaction between two crucial chromatin marks in mammalian tissue homeostasis.
    Keywords:  H3K27 methylation; H3K27M; H3K4 methylation; H3K4M; bivalent genes; differentiation; hematopoiesis; hematopoietic stem and progenitor cells; histone methylation; lysine-to-methionine mutation
    DOI:  https://doi.org/10.1016/j.cell.2025.05.011
  11. Bone Marrow Transplant. 2025 Jun 19.
      In the past decades, treatment for acute myeloid leukemia (AML) has advanced, but allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains vital for improving survival in most patients. This retrospective study, conducted on behalf of the Acute Leukemia Working Party of the EBMT, examines the impact of fludarabine dose in reduced-intensity conditioning regimens on clinical outcomes in patients over 50 years old with AML in first complete remission, without chronic kidney disease. We analyzed 1907 patients who underwent allo-HSCT between 2010 and 2022, stratifying them into four fludarabine dose groups: 110-130 mg/m2, 140-150 mg/m2, 151-160 mg/m2, and 170-190 mg/m2. Our results suggest that a lower fludarabine dose (≤130 mg/m2) is associated with significantly improved leukemia-free survival (LFS), graft-versus-host disease-free/relapse-free survival (GRFS), overall survival, and reduced non-relapse mortality. Multivariate analysis shows that a lower fludarabine dose (≤130 mg/m2) was associated with significantly improved LFS (HR 1.46, 95% CI: 1.09-1.94) and GRFS (HR 1.50, 95% CI: 1.12-1.99). These findings indicate that using a lower fludarabine dosing in older AML patients may improve the efficacy and tolerability of allo-HSCT. Further studies are needed to validate these observations to confirm and expand upon our results, particularly in diverse patient populations and other indications for allo-HSCT.
    DOI:  https://doi.org/10.1038/s41409-025-02614-1
  12. Oncogene. 2025 Jun 18.
      Acute myeloid leukemia (AML) is a heterogeneous clonal disease. Membrane-associated ring-CH type finger 1 (MARCH1), a membrane-anchored E3 ubiquitin ligase, is highly expressed in AML. However, its role in AML remains unclear. Our study showed that MARCH1 expression was strongly associated with FAB classifications and the survival of patients with AML. Gain-of-function and loss-of-function experiments showed that MARCH1 promoted the proliferation of AML cells and inhibited apoptosis and differentiation. In vivo, MARCH1 knockdown inhibited the infiltration of AML cells, resulting in prolonged survival of AML mice. In order to illustrate what cause the high expression of MARCH1, we analyzed the promoter region of MARCH1 and found that POU2F2, a transcription factor with high levels in AML, positively regulated the transcription of MARCH1. Finally, we demonstrated that MARCH1 interacted with MYCT1, a candidate tumor suppressor, and accelerated its ubiquitination and degradation. Remarkably, MYCT1 knockdown abolished the inhibitory effects of MARCH1 knockdown on AML cell growth. Our findings indicate that MARCH1, whose transcription is positively modulated by POU2F2, facilitates the malignant behaviors of AML cells through interacting with MYCT1 and accelerating its ubiquitination and degradation. The results implied that targeting MARCH1 might be a promising therapeutic strategy for AML.
    DOI:  https://doi.org/10.1038/s41388-025-03464-3
  13. Leuk Res. 2025 Jun 05. pii: S0145-2126(25)00092-X. [Epub ahead of print]155 107732
       BACKGROUND: The Janus kinase (JAK) 1/JAK2 inhibitor ruxolitinib has demonstrated efficacy/safety in patients with myelofibrosis; however, not all patients experience optimal and/or stable response, in part owing to dose-limiting toxicities. This phase 2 study evaluated itacitinib (JAK1-selective inhibitor) efficacy/safety alone or combined with low-dose ruxolitinib.
    METHODS: Cohort A received itacitinib 200 mg once daily (QD) plus ruxolitinib at a previous stable dose (≤15 mg total daily dose). Cohort B (previously ruxolitinib-treated) received itacitinib 600 mg QD alone. The primary endpoint was baseline-to-week 24 spleen volume reduction (SVR).
    RESULTS: Twenty-three patients were enrolled (median age, 71.0 years; intermediate-1/-2 risk, 73.9 %; Cohort A, n = 13; Cohort B, n = 10). Mean (standard deviation) percentage SVR from baseline was +6.9 % (27.5 %) and -3.0 % (34.7 %) in Cohorts A and B at week 24 (primary endpoint), and -1.6 % (14.7 %) and -24.6 % (21.7 %) in Cohorts A and B at week 12. SVR from baseline was achieved by 5 and 3 patients in Cohorts A and B at week 24, and by 9 and 7 patients in Cohorts A and B at week 12. Most common treatment-emergent adverse events (TEAEs) were anemia, diarrhea, and fatigue (each n = 8); most common grade ≥ 3 TEAEs were anemia (n = 6), thrombocytopenia (n = 5), fatigue (n = 3), and diarrhea (n = 2).
    CONCLUSIONS: Overall, 8 of 23 patients enrolled achieved SVR at week 24; larger average changes in SVR at week 12 were observed for itacitinib monotherapy vs. the combination. No unexpected safety signals were observed.
    Keywords:  JAK1; JAK2; Janus kinase; Myeloproliferative neoplasm; Phase 2
    DOI:  https://doi.org/10.1016/j.leukres.2025.107732
  14. Blood Neoplasia. 2025 Aug;2(3): 100085
      Intensifying induction by combining venetoclax with a high-dose cytarabine regimen may improve outcomes for high-risk populations such as adult patients with adverse-risk newly diagnosed or relapsed acute myeloid leukemia. In a phase 1 trial testing the novel combination of venetoclax and CLAG-M (cladribine, high-dose cytarabine, granulocyte colony-stimulating factor [G-CSF], and mitoxantrone), the maximum tolerated dose was venetoclax 400 mg on days 1 through 14, combined with cladribine 5 mg/m2 on days 1 through 5, cytarabine 1.5 g/m2 on days 1 through 5, G-CSF 5 μg/kg on days 0 through 5, and mitoxantrone 16 or 18 mg/m2 on days 1 through 3 (for relapsed/refractory and newly diagnosed adverse-risk patients, respectively). The 28-day mortality rate was 5%. Composite complete remission (CR) rate (CR + CR with incomplete hematologic recovery) was 65%. These findings support further phase 2 study of venetoclax in combination with CLAG-M. This trial was registered at www.ClinicalTrials.gov as #NCT04797767.
    DOI:  https://doi.org/10.1016/j.bneo.2025.100085
  15. Br J Haematol. 2025 Jun 16.
      Primary refractory acute myeloid leukaemia (AML) remains a major clinical challenge, with poor outcomes despite salvage chemotherapy. Allogeneic haematopoietic cell transplantation (HCT) continues to be a potentially curative option for these patients. However, recent data on outcomes and prognostic factors specific to primary refractory AML remain limited. We conducted a retrospective analysis of 2600 adult patients with primary refractory AML who underwent their first allogeneic HCT between 2013 and 2022, using data from the Japanese national registry. The 3-year overall survival (OS) and leukaemia-free survival (LFS) rates were 28.5% and 24.4% respectively. The multivariate analysis identified older age (≥50 years), poor performance status (≥2), adverse cytogenetics, extramedullary disease at diagnosis and higher peripheral blood blast count at HCT (≥10%) as significant risk factors for worse OS and LFS. The cumulative incidences of relapse and non-relapse mortality at 3 years were 49.8% and 25.8% respectively. Based on the five significant risk factors, we developed a scoring system that effectively stratified patients into distinct prognostic groups for OS and LFS. This nationwide analysis demonstrated that allogeneic HCT offers the potential for long-term survival in adult patients with primary refractory AML. The proposed risk-scoring system may support clinical decision-making and patient counselling.
    Keywords:  acute myeloid leukaemia; allogeneic haematopoietic cell transplantation; primary induction failure; primary refractory; prognostic score
    DOI:  https://doi.org/10.1111/bjh.20208
  16. J Cell Biol. 2025 Jul 07. pii: e202407141. [Epub ahead of print]224(7):
      The Fanconi anemia (FA) DNA repair pathway is required for the repair of DNA interstrand cross-links (ICLs). ICLs are caused by genotoxins, such as chemotherapeutic agents or reactive aldehydes. Inappropriately repaired ICLs contribute to hematopoietic stem cell (HSC) failure and tumorigenesis. While endogenous acetaldehyde and formaldehyde are known to induce HSC failure and leukemia in FA patients, the effects of other toxic metabolites on FA pathogenesis have not been systematically investigated. Using a metabolism-focused CRISPR screen, we found a synthetically lethal interaction between ALDH9A1 and the deficiency of the FA pathway. Combined deficiency of ALDH9A1 and FANCD2 causes genomic instability, apoptosis, and decreased hematopoietic colony formation. Fanca-/-Aldh9a1-/- mice exhibited an increased incidence of ovarian tumors. A suppressor CRISPR screen revealed that the loss of ATP13A3, a polyamine transporter, resulted in improved survival of FANCD2-/-ALDH9A1-/- cells. These findings nominate high intracellular polyamines and the resulting 3-aminopropanal and acrolein as sources of endogenous DNA damage in patients with FA.
    DOI:  https://doi.org/10.1083/jcb.202407141
  17. Haematologica. 2025 Jun 19.
      The intensity of the conditioning regimen in hematopoietic stem cell transplantation (HSCT) correlates with the risk of relapse, however its potential benefit may be outweighed by the associated risk of toxicity. The addition of total marrow irradiation (TMI) to myeloablative conditioning provides an opportunity to increase intensity with minimal additional toxicity. In this phase 2 clinical trial, 30 patients with high-risk myeloid malignancies received an allogeneic HSCT using myeloablative TMI at 9Gy in combination with standard myeloablative fludarabine/intravenous busulfan (FluBu4) chemotherapy. The study included patients with matched-related donors (n=10) receiving TMI/FluBu4 and patients with matched unrelated (n=14) or 1-antigen mismatched unrelated (n=6) donors receiving TMI/FluBu4 and rabbit antithymocyte globulin. All patients achieved sustained engraftment. Grade 3-4 extramedullary toxicities were: mucositis in 59% (n=17), nausea/vomiting in 10% (n=3) and diarrhea in 7% (n=2) of the patients. Acute graft-versus-host disease (GVHD) grade III-IV was seen in 4 patients (13.3%). Moderate/severe chronic GVHD was observed in 11 patients (36.7%). With a median follow-up of 1483 days (range: 63-2260 days) for patients alive, the overall survival and disease-free survival at 1 year were 72.4% and 65.5%, respectively. GVHD-Free Relapse-Free Survival at 1-year was 41.4%. Of 30 patients in the study, 6 relapsed/progressed (20%) and 5 of them died of the disease (16.7%); whereas 6 patients (20%) died of transplant-related mortality. We conclude that a myeloablative regimen with TMI at 9Gy and FluBu4 was well tolerated and achieved encouraging results in patients with myeloid malignancies at high risk of relapse (clinicaltrials.gov Identifier: NCT03121014).
    DOI:  https://doi.org/10.3324/haematol.2025.287457
  18. EClinicalMedicine. 2025 Jun;84 103283
       Background: Cross sectional studies have demonstrated patients with clonal hematopoiesis of indeterminate potential (CHIP) are at increased risk of developing multiple adverse outcomes, including cytopenia and myeloid neoplasm (MN). One prior study suggests cytopenia or cytosis is a required intermediate step in disease progression from CHIP to MN.
    Methods: We analyzed genomic sequencing data from the NIH All of Us Research Program, Vanderbilt's BioVU repository, and UK Biobank participants (N = 805,249). The study period ranged from 1 January 2006 to 31 December 2023. Genetic mutations, demographic data, laboratory values, and MN outcomes were used to create a case-control study to estimate the risk of incident cytopenia and MN among cases with CHIP and matched controls without CHIP.
    Findings: After applying inclusion and exclusion criteria, the study cohort contained 9374 cases with CHIP and 24,749 matched controls without CHIP. Among the 34,123 participants, 190 (0.56%) developed incident cases of MN and 4151 (12.1%) developed an incident cytopenia. Individuals with CHIP at enrollment who subsequently developed a cytopenia progressed to MN at a rate of 0.5% per year, compared to 0.05% per year for those with CHIP and normal cell counts. Longitudinal analysis across three cohorts demonstrated an increased risk of cytopenia in CHIP patients and identified those at the highest risk of progression. Cytopenia risk factors included smoking (HR = 1.17, 95% CI: [1.05-1.32], P = 5.87 × 10-3), male sex (HR = 1.45, 95% CI: [1.30-1.62], P = 2.17 × 10-11), variant allele frequency ≥0.20 (HR = 1.36, 95% CI: [1.21-1.54], P = 7.56 × 10-7), age ≥65 (HR = 1.41, 95% CI: [1.25-1.57], P = 3.98 × 10-9), mean corpuscular volume ≥100 fL (HR = 2.12, 95% CI: [1.68-2.68], P = 2.58 × 10-10), red cell distribution width ≥15% (HR = 2.59, 95% CI: [2.26-2.98], P = 1.14 × 10-40), mutations in high-risk CHIP genes (HR = 1.48, 95% CI: [1.26-1.75], P = 2.39 × 10-6), and ≥2 CHIP mutations (HR = 1.95, 95% CI: [1.61-2.36], P = 9.73 × 10-12).
    Interpretation: Longitudinal analysis across three large cohorts found that it is rare for patients with CHIP to develop MN without first developing cytopenia. The risk for MN among patients with CHIP resides almost entirely among those with cytopenia. These findings suggest that cytopenia is a critical step in progression from CHIP to MN, underscoring its utility as an endpoint in cancer prevention trials for CHIP patients.
    Funding: National Institutes of Health, Burroughs Wellcome Fund, Edward P. Evans Foundation, Pew Charitable Trusts, Alexander and Margaret Stewart Trust, Beverly and George Rawlings Directorship.
    Keywords:  CCUS; CHIP; Cytopenia; Myeloid neoplasm
    DOI:  https://doi.org/10.1016/j.eclinm.2025.103283
  19. Br J Haematol. 2025 Jun 17.
      The patterns of tryptase normalization, KITD816V clearance and establishment of donor cell chimerism were analysed in 13 patients with systemic mastocytosis and associated haematological neoplasm (AHN) after haematopoietic cell transplantation (HCT). The molecular marker of systemic mastocytosis (SM) (KITD816V) simultaneously disappeared (median + 36 days) with the establishment of donor chimerism (median: +31 days post-HCT; Pearson correlation: -0.99, p < 0.001). In contrast, tryptase normalized median of +228 days after HCT with significant delay to KITD816V clearance (p = 0.01) and full donor chimerism (p = 0.04). Faster normalization was observed after radiation-based conditioning and removal of an infiltrated spleen, while persistence was not associated with relapse of AHN.
    Keywords:   KIT ; HCT; SM‐AHN; allogeneic; reduced intensity conditioning; tryptase
    DOI:  https://doi.org/10.1111/bjh.20211
  20. J Clin Oncol. 2025 Jun 16. JCO2500856
       PURPOSE: Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative treatment for advanced hematologic malignancies. HSCT using human leukocyte antigen (HLA)-mismatched donors is historically associated with inferior survival. Patients from underrepresented racial and ethnic groups more frequently rely on HLA-mismatched donors. We hypothesized that post-transplant cyclophosphamide (PTCy) based graft versus host disease (GVHD) prophylaxis would improve outcomes for HSCT recipients using peripheral blood stem cells (PBSCs) from HLA-mismatched unrelated donors (MMUDs) by reducing the risk of GVHD.
    METHODS: This phase II, nonrandomized, multicenter trial assessed PBSCs in the setting of a GVHD prophylaxis regimen of cyclophosphamide, tacrolimus, and mycophenolate mofetil in two adult strata: myeloablative conditioning (MAC) and reduced-intensity or nonmyeloablative (RIC/NMA) conditioning before HSCT from a MMUD. The primary objective was to estimate 1 year overall survival (OS) for each stratum. Key secondary end points included incidences of acute and chronic GVHD.
    RESULTS: A total of 145 patients enrolled, with 59% self-identifying within an underrepresented group. The 1 year OS was 83.8% (95% CI, 73.1% to 90.4%) for MAC and 78.6% (95% CI, 67% to 86.5%) for RIC/NMA. Incidences of grades III to IV acute GVHD at 6 months were 8% (95% CI, 3.2 to 15.6) for MAC and 10% (95% CI, 4.4 to 18.4) for RIC/NMA. Moderate/severe chronic GVHD at 1 year was 10.3% (95% CI, 4.4 to 18.9) for MAC and 8.6% (95% CI, 3.5 to 16.6) for RIC/NMA. 32% of patients whose donors matched at fewer than seven of eight HLA alleles had similar OS compared with those with donor matched at seven of eight alleles.
    CONCLUSION: PTCy-based GVHD prophylaxis after MMUD HSCT with PBSC grafts results in favorable 1 year OS. Using MMUDs expands donor availability to all patients regardless of ancestry (ACCESS; ClinicalTrials.gov identifier: NCT04904588).
    DOI:  https://doi.org/10.1200/JCO-25-00856
  21. Leuk Lymphoma. 2025 Jun 19. 1-13
      Myelodysplastic neoplasms (MDS) are clonal hematopoietic disorders characterized by ineffective hematopoiesis, dysplasia, and a significant risk of progression to acute myeloid leukemia. Allogeneic hematopoietic stem cell transplant (HSCT) remains the only potentially curative therapy for MDS, particularly for higher-risk disease, but its success depends heavily on the timing of the procedure. This review explores the evolving evidence and decision models guiding the optimal timing of HSCT, balancing the risks of disease progression and transplant-related morbidity and mortality. Key considerations include advancements in disease-specific and transplant-specific risk stratification, such as the IPSS-M and transplant-specific scoring systems, which integrate clinical, cytogenetic, and molecular data to personalize timing decisions. Improvements in haploidentical HSCT and supportive care have expanded the feasibility and safety of HSCT for diverse patient populations, including the elderly. Prospective trials underscore the survival benefits of HSCT over non-transplant approaches for higher-risk MDS, while ongoing studies aim to address uncertainties in pretreatment, post-transplant maintenance therapy, and donor selection. By synthesizing these developments, this review provides practical insights into optimizing HSCT timing to improve outcomes for MDS patients.
    Keywords:  Myelodysplastic syndrome; allogeneic stem cell transplantation; comorbidity score; graft versus host disease; timing
    DOI:  https://doi.org/10.1080/10428194.2025.2514662
  22. Lancet Haematol. 2025 Jun 13. pii: S2352-3026(25)00144-9. [Epub ahead of print]
       BACKGROUND: Two doses of subcutaneous blinatumomab in patients with relapsed or refractory B-cell acute lymphoblastic leukaemia were identified as preliminary recommended phase 2 doses, based on the dose-escalation phase of this multicentre single-arm, phase 1/2 trial. Here, we aim to further study the safety, activity, and pharmacokinetics of these doses in all participants who have received them, including those treated in the completed phase 1b expansion part of the study.
    METHODS: We did a post-hoc analysis of data from patients enrolled in the dose-escalation and dose-expansion phases and in the pharmacokinetic evaluation cohort of this multicentre, single-arm, phase 1/2 study. Patients were recruited from 44 hospitals in 11 countries. Eligible participants were aged 18 years or older with relapsed or refractory B-cell acute lymphoblastic leukaemia, at least 5% of blasts in the bone marrow, and an Eastern Cooperative Oncology Group performance status of 2 or lower. Patients received either 250 μg subcutaneous blinatumomab once daily in week 1 of cycle 1 and then 500 μg three times weekly thereafter (250 μg/500 μg group), or 500 μg and then 1000 μg subcutaneous blinatumomab on the same schedule (500 μg/1000 μg group), previously identified as the preliminary recommended phase 2 doses. Each treatment cycle included a 4-week treatment period and a 1-week treatment-free interval. Patients received between two and five cycles. The primary endpoint for the dose-expansion phase was complete remission or complete remission with partial haematological recovery within the first two cycles, which was used as the primary outcome for this study. Data were pooled from all cohorts of the same dose level to form two dose groups. The response rates, adverse event incidence, and pharmacokinetics were summarised in each dose group separately and compared descriptively. Response was calculated with two-sided exact 80% CIs (Clopper-Pearson method). This study is registered with ClinicalTrials.gov, NCT04521231; phase 1 is complete, and phase 2 is active but not recruiting.
    FINDINGS: Participants were recruited from Oct 18, 2021, to Sept 23, 2024, and median follow-up for the analyses was 5 months (IQR 3-9). Of the 88 patients included in the analysis at the data cutoff of Nov 28, 2024, 36 (41%) were treated with the 250 μg/500 μg regimen and 52 (59%) with the 500 μg/1000 μg regimen. The enrolled population comprised 55 (63%) male and 33 (38%) female participants; 56 (64%) were White, six (7%) Asian, three (3%) Black or African American, two (2%) American Indian or Alaska Native, and 20 (23%) other. Hispanic or Latino ethnicity was reported for 33 (38%) patients. 27 (75%) of 36 patients in the 250 μg/500 μg group and 41 (79%) of 52 in the 500 μg/1000 μg group showed complete remission or complete remission with partial haematological recovery. The most common grade 3-4 adverse events were neutropenia (19 [22%] patients), cytokine release syndrome (CRS; 18 [20%] patients), and immune effector cell-associated neurotoxicity syndrome (ICANS; 15 [17%] patients). Serious adverse events occurred in 70 (80%) of 88 patients and included CRS (33 [38%] patients), ICANS (20 [23%] patients), and neurotoxicity (six [7%] patients). No treatment-related deaths were reported. Consistent pharmacokinetics with dose-proportional exposures was observed following subcutaneous administration. Based on the totality of data, including efficacy, safety, and pharmacokinetic data, the subcutaneous blinatumomab dose regimen of 250 μg/500 μg was selected as the recommended phase 2 dose.
    INTERPRETATION: Treatment with subcutaneous blinatumomab at the two dose regimens of 250 μg/500 μg and 500 μg/1000 μg resulted in promising preliminary activity and a manageable safety profile in adults with relapsed or refractory B-cell acute lymphoblastic leukaemia. The phase 2 part of the trial is ongoing to further evaluate subcutaneous blinatumomab activity and duration of response.
    FUNDING: Amgen.
    DOI:  https://doi.org/10.1016/S2352-3026(25)00144-9
  23. Ann Hematol. 2025 Jun 14.
      The current retrospective study evaluated clinical, genetic, and prognostic correlates of increased absolute basophil (ABC) and eosinophil (AEC) counts in polycythemia vera (PV; N = 475) and essential thrombocythemia (ET; N = 658). Median (range) ABC and AEC were 0.1 (0-3.2) and 0.3 (0-6.5) x 109/L in PV and 0.07 (0-0.8) and 0.2 (0-1.4) x 109/L in ET. In PV, ABC ≥ 0.1 × 10⁹/L was associated with palpable splenomegaly and increased AEC, leukocyte count, and platelet count while AEC ≥ 0.5 × 10⁹/L was associated with increased ABC and leukocyte count. In ET, ABC ≥ 0.1 × 10⁹/L was associated with increased AEC, leukocyte count, platelet count, and cardiovascular risk factors while AEC ≥ 0.5 × 10⁹/L correlated with ABC and increased leukocyte count; genetic associations were seen only in ET and included ABC ≥ 0.1 × 10⁹/L with triple-negative driver mutation status (p = 0.03). In PV, AEC did not correlate with overall (OS), leukemia-free (LFS), myelofibrosis-free (MFFS), arterial thrombosis-free (ATFS), or venous thrombosis-free (VTFS) survival; by contrast, ABC ≥ 0.1 × 10⁹/L was associated with longer ATFS (p = 0.03) while ABC ≥ 0.3 × 10⁹/L was associated with inferior LFS (p < 0.01) and MFFS (p < 0.01); the associations with LFS and MFFS were sustained during multivariable analysis. In ET, both ABC ≥ 0.1 × 10⁹/L and AEC ≥ 0.5 × 10⁹/L were independently associated with inferior OS but impact on LFS, MFFS, ATFS, or VTFS was not apparent. The results from the current study warrant additional studies to clarify the potential association between basophilia in PV and disease transformation into acute myeloid leukemia and myelofibrosis.
    Keywords:  Basophil; Eosinophil; Leukemia; Myelofibrosis; Survival
    DOI:  https://doi.org/10.1007/s00277-025-06422-x
  24. Cell Rep Med. 2025 Jun 06. pii: S2666-3791(25)00265-4. [Epub ahead of print] 102192
      KAT6A is a histone acetyltransferase that is emerging as a therapeutic target in cancer, including estrogen receptor-positive (ER+) breast cancer. Here, we perform CRISPR screens to identify the chromatin adaptor Menin as a regulator of KAT6A/B inhibitor response. Co-treatment with KAT6A/B and Menin inhibitors has synergistic anti-proliferative effects in ER+, but not ER-, breast cancer lines. Our data reveal that KAT6A and Menin-KMT2A cooperatively regulate ER-driven gene expression via direct effects on ESR1 expression and co-localization at ER target genes. Combined KAT6A/B and Menin inhibition displaces KAT6A and Menin-KMT2A from promoters of ER-driven genes leading to selective RNA polymerase II chromatin loss at these loci. Importantly, combined KAT6A/B and Menin inhibition is effective in ER+ patient-derived xenograft models and in multiple models of endocrine resistance. KAT6A/B and Menin inhibitors are currently in clinical trials and have shown manageable toxicity profiles, underscoring the potential therapeutic relevance for ER+ breast cancer.
    Keywords:  ER; KAT6A; Menin; breast cancer; chromatin; estrogen receptor
    DOI:  https://doi.org/10.1016/j.xcrm.2025.102192
  25. Blood Cancer Discov. 2025 Jun 17. OF1-OF9
      Clonal hematopoiesis of indeterminate potential (CHIP) has broad clinical relevance, and TP53 plays various roles within cells. However, the gene-specific and cross-disease significance of CHIP with TP53 mutations (TP53-CHIP) remains unclear. In this study, we evaluated TP53-CHIP using targeted sequencing data of 140,597 individuals without hematologic neoplasms in BioBank Japan. We identified 1,157 individuals with TP53-CHIP and clarified the characteristics of mutations and carriers. TP53-CHIP was associated with poor overall survival, especially because of lymphoid neoplasms and respiratory disease, in addition to myeloid neoplasms. Significant interactions accompanied by excess risks were observed between TP53-CHIP and lifestyle factors for disease-specific mortality: acetaldehyde exposure (resulting from the interaction between drinking and the germline variant of ALDH2) for myeloid neoplasms and smoking for respiratory disease. The clinical significance of TP53-CHIP was sometimes largely independent of variant allele fractions. These findings elucidate aspects of disease pathogenesis and inform personalized risk management.
    SIGNIFICANCE: TP53-CHIP contributed to a wide range of outcomes besides myeloid neoplasm mortality. TP53-CHIP, when combined with environmental factors, showed a remarkably higher risk for disease-specific mortality, accompanied by excess risks.
    DOI:  https://doi.org/10.1158/2643-3230.BCD-24-0355
  26. Br J Haematol. 2025 Jun 19.
      Paediatric mastocytosis is a heterogeneous disorder with different clinical subtypes and an often indolent disease course. The molecular landscape of genetic mutations, beyond KIT D816V, remains under exploration. We thus investigated the prevalence of myeloid genetic mutations in peripheral blood samples of 69 paediatric patients with cutaneous mastocytosis and systemic mastocytosis (SM) using next-generation sequencing. KIT D816V was exclusively found in those with SM, while other KIT mutations (D419del, A502_Y503dup, Y503_A507dup, G565V and N822H) were only detected in those with cutaneous disease. Although no other common non-KIT myeloid mutations were shared among patients, we identified 64 non-synonymous coding genetic mutations across 30 genes, including 15 classified as pathogenic/likely pathogenic, with none occurring in SRSF2 and ASXL1. Most of these pathogenic/likely pathogenic non-KIT mutations (86.7%) were found in paediatric patients with SM. In summary, we discovered a number of non-KIT myeloid alterations in paediatric mastocytosis and which were more common in children with systemic disease. Variant allele frequency of many of these gene alterations was about 50% or 100%, suggesting germline in nature. The long-term impact of these additional genetic alterations on disease course is uncertain at present and supports the need for assessment in long-term follow-up studies.
    Keywords:  mastocytosis; mutations; next‐generation sequencing; paediatrics
    DOI:  https://doi.org/10.1111/bjh.20214
  27. Cancer Res. 2025 Jun 18.
      XPO1 is a nuclear export receptor that is essential for cell survival. Previous genomic analyses have identified recurrent XPO1 hotspot mutations in cancer. Here, we conducted a large-scale genomic analysis of 217,570 cancer patients to identify and characterize XPO1 variants from real-world patient tumors. XPO1 harbored a R749Q mutation in various solid tumors, with a clear enrichment in endometrial and colorectal cancers, and XPO1R749Q mutations significantly co-occurred with POLE mutations. Analysis of isogenic colon cancer cell lines revealed that XPO1R749Q localized more in the cytoplasm than wildtype XPO1, with enhanced export of a large number of proteins. Structural modeling of XPO1R749Q suggested an increase in RanGTP affinity, which is consistent with enhanced protein export. A compound library screen using over 200 FDA-approved anticancer drugs indicated a general trend towards chemoresistance, specifically to topoisomerase I inhibition, in XPO1R749Q mutant cells. Mechanistically, XPO1R749Q mutant cells exhibited enhanced DNA damage response via RPA phosphorylation in response to topoisomerase I inhibition. Combining XPO1 and topoisomerase I inhibitors reduced DNA damage-induced RPA phosphorylation and mediated synergistic antitumor effects in cells harboring the XPO1R749Q mutation. Finally, the combination of selinexor and irinotecan overcame chemotherapeutic resistance in xenograft mouse models, prolonging survival. These findings suggest that XPO1 alterations in cancer are selected for in POLE mutant tumors and may confer resistance to DNA-damaging chemotherapies, which have implications for patients with tumors bearing XPO1R749Q and for XPO1 inhibitor development in cancer.
    DOI:  https://doi.org/10.1158/0008-5472.CAN-24-3112
  28. Exp Hematol. 2025 Jun 14. pii: S0301-472X(25)00112-2. [Epub ahead of print] 104821
      Transcription is regulated in a multitude of ways to ensure lineage- and context-specific gene expression in a co-ordinated fashion. Haematopoiesis is an exemplar process for studying the mechanisms of tightly regulated activation and repression of gene expression programmes by transcription and gene regulatory complexes. These complexes act by post-translational modification of histones and non-histone proteins, epigenetic modifications of DNA, ATP-dependent chromatin remodelling, scaffolding and recruitment of combinatorial protein complexes and alteration of three-dimensional genome conformation to bring about lineage-specific gene expression. This review focusses on the function of these gene regulatory complexes in haematopoiesis and how they are hijacked in Acute Myeloid Leukaemia, highlighting therapeutic progress and opportunities.
    DOI:  https://doi.org/10.1016/j.exphem.2025.104821
  29. Blood Rev. 2025 Jun 11. pii: S0268-960X(25)00062-1. [Epub ahead of print] 101317
      The prognosis of patients with AML varies significantly with age, driven by biological heterogeneity and age-associated factors such as comorbidities, functional status, and hospitalization burden. Many novel therapies have been approved in recent years; however, pivotal trials often include patients within a restricted age range, limiting the extrapolation of their findings across the broader AML population. For example, the FLT3 inhibitor midostaurin was added to chemotherapy for patients aged 18-60 years, while the BCL-2 inhibitor venetoclax was combined with azacitidine in patients aged 75 years and older, leaving important knowledge gaps regarding their efficacy and safety in other age groups. Moreover, for several novel therapies, particularly in populations outside the original trial age range, supporting evidence is derived primarily from single-arm studies or real-world experience rather than randomized controlled trials, further complicating clinical decision-making. This review explores the efficacy and safety of widely used traditional and novel therapies for AML, with particular focus on the impact of age on these different therapeutic regimens.
    Keywords:  AML; Acute myeloid leukemia; Age-related outcomes; Novel therapies; Targeted therapy
    DOI:  https://doi.org/10.1016/j.blre.2025.101317