Publications by authors named "B Lioure"
Article Synopsis
- The study examines the blood-related characteristics and overall prognosis of 127 patients with telomere biology disorders (TBD) who were diagnosed after age 15, highlighting a lack of data on this topic.
- At diagnosis, significant haematological issues were present in nearly 76% of patients, with bone marrow failure (BMF) being the most common, affecting 46.5% of the cases, while some patients also developed additional complications over time.
- The findings suggest that BMF patients tend to be younger and have a better survival rate compared to those with higher-risk blood cancers, indicating TBD as a complex multi-organ disease needing further research on its evolutionary nature and outcomes.
Article Synopsis
- RUNX1 is crucial for human blood formation and is linked to various mutations, particularly in acute myeloid leukemia (AML), leading the WHO to create a category for mutations in RUNX1 in 2016.
- A recent study examined 32 AML patients at Strasbourg University Hospital to explore features related to RUNX1 mutations and the presence of plasmacytoid dendritic cells (pDC).
- The findings indicated that AML cases with RUNX1 mutations don't show consistent characteristics and are not a separate AML subtype, with some fitting the criteria for a recently recognized pDC-related AML according to the 2022 WHO classification.
Article Synopsis
- The study examines outcomes of 67 patients (average age 20.6 years) who underwent their first allogeneic hematopoietic stem cell transplant (HSCT) due to GATA2 deficiency across 21 centers in June 2022, showing varied indications for the transplant.
- The findings reveal significant rates of acute graft versus host disease (GvHD) and chronic GvHD; the incidence of relapses was low, with overall survival rates at 1 and 5 years being 83% and 72%, respectively.
- The analysis highlights that monitoring bone marrow for clonal evolution is crucial to initiate HSCT before the development of excessive blasts, noting that factors like earlier years of HSCT and certain
Hematopoietic stem cell transplantation (HSCT) for severe ADs was developed over the past 25years and is now validated by national and international medical societies for severe early systemic sclerosis (SSc) and relapsing-remitting multiple sclerosis (MS) and available as part of routine care in accredited center. HSCT is also recommended, with varying levels of evidence, as an alternative treatment for several ADs, when refractory to conventional therapy, including specific cases of connective tissue diseases or vasculitis, inflammatory neurological diseases, and more rarely severe refractory Crohn's disease. The aim of this document was to provide guidelines for the current indications, procedures and follow-up of HSCT in ADs.
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