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Summary: European LeukemiaNet refined their risk classification of acute myeloid leukaemia (AML) in 2022 (ELN 2022) according to the two new myeloid classifications published the same year. We have retrospectively assessed the prognostic value of the ELN 2022 in 120 AML patients undergoing allogeneic haematopoietic cell transplantation (allo‐HCT), including 99 in first complete response (CR1) from 2011 to 2021 in our centre. Adverse risk patients (Adv) presented inferior outcome in terms of overall survival (OS) and leukaemia‐free survival (LFS) (OS [p = 0.003], LFS [p = 0.02]), confirmed in multivariate analysis (hazard ratio [HR] for OS = 2.00, p = 0.037). These results were also seen in patients allografted in CR1. Further analysis identified a subgroup named adverse‐plus (AdvP), including complex karyotype, MECOM(EVI1) rearrangements and TP53 mutations, with worse outcomes than the rest of groups of patients, including the Adv (HR for OS: 3.14, p < 0.001, HR for LFS: 3.36, p < 0.001), with higher 2‐year cumulative incidence of relapse (p < 0.001). Notably, within this analysis, the outcome of Adv and intermediate patients were similar. These findings highlight the prognostic value of ELN 2022 in patients undergoing allo‐HCT, which can be improved by the recognition of a poor genetic subset (AdvP) within the Adv risk group. [ABSTRACT FROM AUTHOR]

Summary: Recently modified diagnostic criteria for chronic myelomonocytic leukaemia (CMML) have lowered the cut‐off for absolute monocytosis. In the largest series to date, we have analysed 313 CMML patients, including 104 with oligomonocytic (OM)‐CMML. Five‐year survival was longer for OM‐CMML than for other patients (p < 0.001). Multivariate analysis identified OM‐CMML as a favourable prognostic factor (HR 0.58; p = 0.002). The 5‐year cumulative incidence of progression to classical CMML was 47%. Older age and transfusion dependence were adverse prognostic factors for OM‐CMML. Our results support the inclusion of OM‐CMML in the CMML category as a subtype with superior outcomes. [ABSTRACT FROM AUTHOR]

Summary: Acute myeloid leukaemia (AML) with t(6;9)(p23;q34) is a poor‐risk entity, commonly associated with FLT3‐ITD (internal tandem duplication). Allogeneic stem‐cell tranplantation (allo‐SCT) is recommended, although studies analysing the outcome of allo‐SCT in this setting are lacking. We selected 195 patients with t(6;9) AML, who received a first allo‐SCT between 2000 and 2016 from the EBMT (European Society for Blood and Marrow Transplantation) registry. Disease status at time of allo‐SCT was the strongest independent prognostic factor, with a two‐year leukaemia‐free survival and relapse incidence of 57% and 19% in patients in CR1 (first complete remission), 34% and 33% in CR2 (second complete remission), and 24% and 49% in patients not in remission, respectively (P < 0·001). This study, which represents the largest one available in t(6;9) AML, supports the recommendation to submit these patients to allo‐SCT in CR1. [ABSTRACT FROM AUTHOR]

The article presents several case studies related to early T-cell precursor lymphoblastic leukaemia. It discussed the conditions of patients including a 31-year-old man, presented with a mediastinal mass and white blood cell (WBC) count, without central nervous system (CNS) involvement; a 34-year-old man who presented with pancytopenia; and a 39-year-old man, presented with a mediastinal mass and presence of 0.6 percent leukaemic cells.

VEXAS syndrome is a haemato-inflammatory disease caused by somatic UBA1 mutations and characterized by cytoplasmic vacuoles in myeloid and erythroid precursor cells. Although there is currently no standard treatment algorithm for VEXAS, patients are generally treated with anti-inflammatory therapies focused on symptom management, with only partial effectiveness. Hypomethylating agents (HMA) have shown promise in VEXAS patients with concomitant myelodysplastic syndrome (MDS), while the efficacy of HMA in VEXAS patients without MDS is largely unknown. Furthermore, the usefulness of monitoring the variant allele frequency (VAF) of UBA1 or vacuolization in precursor cells over the course of treatment has not been extensively investigated. We have evaluated the efficacy of HMA in four VEXAS patients without MDS and performed longitudinal analyses of the VAF of UBA1 and vacuolization during treatment. HMA treatment led to overall clinical improvement, a dramatic reduction in the VAF of UBA1, normalization of haematological and inflammatory markers and a quantifiable decrease in vacuolization, leading us to speculate that unlike anti-inflammatory therapies, HMA may well act as a disease-modifying treatment. If these findings are confirmed in further studies, it could lead to the early use of HMA in the treatment of all VEXAS patients-with or without MDS., (© 2025 British Society for Haematology and John Wiley & Sons Ltd.)