Publications by authors named "Bart J Biemond"
Purpose: To evaluate the presence and progression of maculopathy in patients with sickle cell disease (SCD) using Optical Coherence Tomography (OCT) and OCT-Angiography (OCTA), and to identify clinical/laboratory risk factors for progression during follow-up.
Methods: Complete ophthalmic examination, including fundoscopy and macular SD-OCT/OCTA scans, was performed in consecutive SCD-patients (HbSS/HbSβ0/HbSβ+/HbSC genotype) during baseline and follow-up visits. SCR stage was based on fundoscopy instead of the Goldberg classification, since fluorescein angiography was not routinely used.
Background: Relapsed acute myeloid leukemia (AML) and high-risk myelodysplastic syndrome (HR-MDS) are associated with a poor prognosis. It is unknown which re-induction therapy provides the highest chance of durable remission. Commonly used therapies are high dose cytarabine (HiDAC) and triple therapy consisting of fludarabine, cytarabine, and idarubicin combined with granulocyte colony-stimulating factor (FLAG-IDA).
View Article and Find Full Text PDFArticle Synopsis
- Sickle cell retinopathy (SCR) is a serious eye condition linked to sickle cell disease (SCD) that can severely affect vision, prompting recommendations for screening.
- A scoping review analyzed 67 studies to identify demographic, genetic, and hematological risk factors for SCR and sickle cell maculopathy (SCM), highlighting key risks such as older age, male sex, and specific genotypes.
- While screening is essential, further research is necessary to understand the effectiveness of treatments for SCR and the long-term impacts of SCM on patients.
Hemostatic conditions following autologous transfusion of fresh vs stored platelets in experimental endotoxemia: an open-label randomized controlled trial with healthy volunteers.
Res Pract Thromb Haemost
November 2024
Article Synopsis
- The study aimed to investigate how fresh and stored platelet concentrates (PCs) behave during inflammation, specifically in a controlled human model of endotoxemia induced by lipopolysaccharide (LPS).
- Researchers found that LPS increased markers related to coagulation and clotting, leading to a generally lower clotting ability in subjects compared to controls, while platelet transfusions improved clotting time and strength in those with endotoxemia.
- Despite these improvements in coagulation, there were no significant differences noted between the effects of fresh versus stored platelets on coagulation parameters like thrombin-antithrombin complex (TATc) or extracellular vesicles (EVs).
Article Synopsis
- Silent cerebral infarcts (SCIs) are common in patients with severe forms of sickle cell disease (SCD) and thalassemia, although the underlying causes are not fully understood.
- This study compared cerebral blood flow, oxygen metabolism, and SCI lesions in severe and mild SCD patients, thalassemia patients, and healthy controls before and after a vasodilatory challenge.
- Findings showed that while severe SCD and thalassemia patients had higher blood flow, their oxygen consumption was lower; this suggests that anemia rather than cerebral blood flow may play a key role in the development of SCIs, especially in milder forms of SCD.
Sickle cell disease (SCD) is complicated by silent cerebral infarcts (SCIs), for which anemia is an important risk factor. Despite normal oxygen delivery (OD), cerebral vascular reserve (CVR), and cerebral metabolic rate of oxygen (CMRO) are diminished in SCD, possibly causing the formation of SCIs. Voxelotor inhibits polymerization by increasing the hemoglobin oxygen binding, ameliorating hemolytic anemia.
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- - The study focuses on older patients (over 60 years) with acute myeloid leukemia (AML) and aims to create a prognostic model to identify those who would benefit from intensive chemotherapy and stem cell transplantation.
- - Researchers analyzed data from 1,910 patients, using a random survival forest to evaluate clinical and genetic factors associated with overall survival, resulting in the identification of four distinct survival groups based on nine variables.
- - The newly developed AML60+ classification shows improved prognostic accuracy and indicates that intermediate- and very poor-risk patients may benefit from allogeneic hematopoietic cell transplantation, potentially guiding treatment decisions for older adults with AML.
Article Synopsis
- * A total of 249 eyes from 137 patients were examined; results showed significant macular thinning and variations in vessel density, though no clear association with the severity of SCR.
- * Despite the prevalence of macular issues, they did not lead to visual impairment, and OCTA imaging proved ineffective in predicting SCR risk in these patients.
Given the selection of elderly patients with AML in first complete remission (CR1) the advantage of consolidation with allogeneic hematopoietic cell transplantation (HCT) over chemotherapy is still unclear. Newly diagnosed AML patients in CR1 aged 60-75 years were registered and a donor search initiated. After one consolidation cycle, patients with a matched donor were randomized to HCT with fludarabine/low-dose total body irradiation and cyclosporine/mycophenolate mofetil immunosuppression or conventional non-HCT.
View Article and Find Full Text PDFBackground: Reported bleeding incidences following central venous catheter (CVC) placement highly depend on methods of bleeding assessment. To determine the direction and magnitude of the bias associated with retrospective data collection, we used data from the PACER randomized controlled trial and a previous retrospective cohort study.
Study Design And Methods: A patient-level comparison of CVC-related bleeding severity was made among (1) the prospectively collected clinical bleeding assessment of the PACER trial, (2) centralized assessment of CVC insertion site photographs, and (3) retrospective chart review.
Mitapivat is an investigational, oral, small-molecule allosteric activator of pyruvate kinase (PK). PK is a regulatory glycolytic enzyme that is key in providing the red blood cell (RBC) with sufficient amounts of adenosine triphosphate (ATP). In sickle cell disease (SCD), decreased 2,3-DPG levels increase the oxygen affinity of hemoglobin, thereby preventing deoxygenation and polymerization of sickle hemoglobin.
View Article and Find Full Text PDFObjectives: To develop and validate a prediction model for 1-year mortality in patients with a hematologic malignancy acutely admitted to the ICU.
Design: A retrospective cohort study.
Setting: Five university hospitals in the Netherlands between 2002 and 2015.
Nonmyeloablative, matched sibling donor hematopoietic stem cell transplantation with alemtuzumab/total body irradiation (TBI) conditioning is a curative therapy with low toxicity for adults with sickle cell disease (SCD). However, relatively low donor chimerism levels and graft rejection remain important challenges. We hypothesized that adding azathioprine/hydroxyurea preconditioning will improve donor chimerism levels and reduce graft failure rate.
View Article and Find Full Text PDFBackground: We recently reported results of the prospective, open-label HOVON-100 trial in 334 adult patients with acute lymphoblastic leukemia (ALL) randomized to first-line treatment with or without clofarabine (CLO). No improvement of event-free survival (EFS) was observed, while a higher proportion of patients receiving CLO obtained minimal residual disease (MRD) negativity.
Aim: In order to investigate the effects of CLO in more depth, two multi-state models were developed to identify why CLO did not show a long-term survival benefit despite more MRD-negativity.
Article Synopsis
- Sickle cell disease can cause serious issues with organs, making patients very sick, so researchers wanted to see if a treatment called stem cell transplantation helps or hurts organ function.
- They looked at many studies and found that while some organ functions got better after the treatment, like the spleen and brain blood flow, not all problems went away, and some got worse, especially in kids.
- Overall, while stem cell transplantation can help certain organ issues in sickle cell disease patients, it can also cause new problems, so more research is needed to find out who would benefit the most from it.
Factors influencing the activation of neutrophils in SCD and the potential neutrophil-mediated ameliorating effects of therapies in SCD.
View Article and Find Full Text PDFTargeting the primary pathogenic event of sickle cell disease (SCD), the polymerization of sickle hemoglobin (HbS), may prevent downstream clinical events. Mitapivat, an oral pyruvate kinase (PK) activator, has therapeutic potential by increasing adenosine triphosphate (ATP) and decreasing 2,3-diphosphoglycerate (2,3-DPG), a glycolytic red blood cell (RBC) intermediate. In the previously reported 8-week dose-finding period of this phase 2, investigator-initiated, open-label study, mitapivat was well tolerated and showed efficacy in SCD.
View Article and Find Full Text PDFSickle cell disease (SCD) is characterized by chronic hemolytic anemia associated with impaired cerebral hemodynamics and oxygen metabolism. Hematopoietic stem cell transplantation (HSCT) is currently the only curative treatment for patients with SCD. Whereas normalization of hemoglobin levels and hemolysis markers has been reported after HSCT, its effects on cerebral perfusion and oxygenation in adult SCD patients remain largely unexplored.
View Article and Find Full Text PDFIntroduction: Neutrophils promote chronic inflammation and release neutrophil extracellular traps (NETs) that can drive inflammatory responses. Inflammation influences progression of sickle cell disease (SCD), and a role for NETs has been suggested in the onset of vaso-occlusive crisis (VOC). We aimed to identify factors in the circulation of these patients that provoke NET release, with a focus on triggers associated with hemolysis.
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