Introduction: Immunosuppressive therapy (IST) with antithymocyte globulin (ATG) and ciclosporin is standard of care for patients with severe aplastic anaemia (sAA) not eligible or suitable for allogeneic stem cell transplant. While patients respond to IST, few achieve complete responses and a significant proportion are refractory or relapse. The addition of eltrombopag, a thrombopoietin-receptor agonist (TPO-A), to IST has been shown to improve haematological responses in sAA.
View Article and Find Full Text PDFBest Pract Res Clin Haematol
December 2023
The bone marrow failure syndromes (BMFS) are a diverse group of acquired and inherited diseases which may manifest in cytopenias, haematological malignancy and/or syndromic multisystem disease. Patients with BMFS frequently experience poor outcomes, and improved treatment strategies are needed. Collation of clinical characteristics and patient outcomes in a national disease-specific registry represents a powerful tool to identify areas of need and support clinical and research collaboration.
View Article and Find Full Text PDFObjective: Recommendations for perioperative management of direct oral anticoagulant (DOAC) treatment in cardiac surgery are lacking. To establish a standardized approach for these patients, we compared hemorrhagic complications and clinical outcomes in patients on DOAC medication, patients on vitamin K antagonists (VKA), and patients without preoperative anticoagulation.
Methods: All 3 groups underwent major cardiac surgery and were retrospectively analyzed: patients on DOAC were advised to take their last DOAC dose 4 days before hospital admission, and DOAC plasma levels were measured the day before surgery.
Objectives: The three direct oral anticoagulants (DOACs) dabigatran, apixaban and rivaroxaban are now widely used in clinical practice. For patients requiring perioperative interruption of DOACs, heparin bridging is still under discussion. Here we show, for the first time, the influence of concomitantly used DOACs and heparins on laboratory assays.
View Article and Find Full Text PDFDevelopmental biology aims to understand how the dynamics of embryonic shapes and organ functions are encoded in linear DNA molecules. Thanks to recent progress in genomics and imaging technologies, systemic approaches are now used in parallel with small-scale studies to establish links between genomic information and phenotypes, often described at the subcellular level. Current model organism databases, however, do not integrate heterogeneous data sets at different scales into a global view of the developmental program.
View Article and Find Full Text PDFDevelopmental- and tissue-specific expression of globin genes is mediated by a few key elements within the proximal promoter of each gene. DNA-binding assays previously identified NF-Y, GATA-1, C/EBPbeta and C/EBPgamma as candidate regulators of beta-globin transcription via the CCAAT-box, a promoter element situated between CACC- and TATA-boxes. We have identified C/EBPdelta as an additional beta-globin CCAAT-box binding protein.
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