A new strategy for monitoring of direct oral anticoagulants in patients with cyanotic and complex congenital heart disease.

Background: Patients with congenital heart disease (CHD) often require an oral anticoagulation. Vitamin K antagonists (VKA) are the standard treatment, however, an increased hematocrit in patients with secondary erythrocytosis due to cyanosis complicates the correct measurement of the international normalized ratio. Direct oral anticoagulants (DOAC) could be an alternative, but data on their efficacy and safety in complex and cyanotic CHD patients are scarce.

Biotinylation of human platelets is compatible with pathogen inactivation treatment and cold storage for clinical studies.

Background: Production of platelet concentrates (PCs) involves several steps that significantly affect platelet behavior. To gain a deeper understanding of how storage conditions impact donor platelet recirculation and functionality post-transfusion, ex vivo platelet labeling is a feasible approach. However, before pursuing clinical investigations of platelet recirculation and function in humans, we aimed to determine the effects of pathogen inactivation technology (PIT) and storage conditions (4°C vs.

[Sickle cell crisis: inpatient management].

Sickle cell anemia is the world's most common monogenic disease, with a steady rise in the number of cases also in Switzerland. It presents as regenerative anemia and recurrent vaso-occlusive episodes, which can impact all organs. Acute and chronic complications increase morbidity and mortality, significantly reducing life expectancy compared to the general population.

Targeted management of coexistent severe thrombophilias-A case report of a successful pregnancy despite paroxysmal nocturnal hemoglobinuria and hereditary protein C deficiency.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematological disorder characterized by the absence of complement regulatory proteins on the surface of erythrocytes, leading to intravascular hemolysis and thrombosis. Managing PNH during pregnancy poses significant challenges due to increased risks of morbidity and mortality. This case report describes the detailed obstetric course of a 44-year-old woman with PNH and additional hereditary protein C deficiency who had previously experienced multiple thrombotic events and adverse pregnancy outcomes (two early miscarriages and one stillbirth at 25 weeks gestation [WG]), treated with eculizumab (terminal C5 inhibitor) and optimal anticoagulation management.

Mepacrine Flow Cytometry Assay for the Diagnosis of Platelet δ-granule Defects: Literature Review on Methods-Towards a Shared Detailed Protocol.

Accurate assessment of platelet secretion is essential for the diagnosis of inherited or acquired platelet function disorders and more specifically in identifying δ-storage pool disease. Mepacrine, a fluorescent dye, specifically accumulates in platelet δ-granules. The mepacrine flow cytometry (mepacrine FCM) assay has been used for more than half a century in the clinical laboratory as a diagnostic tool for platelet δ-granule disorders.

Direct oral anticoagulants in cirrhosis: Rationale and current evidence.

Cirrhosis is a major health concern worldwide with a complex pathophysiology affecting various biological systems, including all aspects of haemostasis. Bleeding risk is mainly driven by portal hypertension, but in end-stage liver disease it is further increased by alterations in haemostatic components, including platelet function, coagulation, and fibrinolysis. Concurrently, patients with cirrhosis are prone to venous thromboembolic events (VTE) because of the altered haemostatic balance, in particular an increase in thrombin generation.

Dabigatran accumulation in acute kidney injury: is more better than less to prevent bleeding? A case report.

Dabigatran is an oral anticoagulant that is mainly renally excreted. Despite its efficacy in preventing thromboembolic events, concerns arise regarding bleeding complications in patients with acute kidney injury. Idarucizumab is its specific antidote and reverses quickly and effectively dabigatran anticoagulation effects in situations of severe bleeding or pending surgical procedures, but its benefit beyond these two indications remains uncertain.

Global coagulation assays detect an early prothrombotic state in children with acute lymphoblastic leukemia.

Background: Pediatric patients with acute lymphoblastic leukemia (ALL) are at highest risk of venous thromboembolism during the induction therapy (IT). These events are not predictable by conventional coagulation assays.

Objectives: To investigate the utility of global coagulation assays (GCAs) for assessing the hemostatic state in children with ALL during IT.

Increase of Phosphoprotein Expressions in Amotosalen/UVA-Treated Platelet Concentrates.

Background: Pathogen inactivation treatment (PIT) has been shown to alter platelet function, phenotype, morphology and to induce a faster aging of platelet concentrates (PCs). Key pieces of information are still missing to understand the impacts of PITs at the cellular level.

Objectives: This study investigated the impact of amotosalen/UVA on PCs, from a post-translational modifications (PTM) point of view.

Inhibition of mitochondrial calcium transporters alters adp-induced platelet responses.

Introduction: ADP-stimulated elevation of cytosolic Ca is an important effector mechanism for platelet activation. The rapidly elevating cytosolic Ca is also transported to mitochondrial matrix via Mitochondrial Ca Uniporter (MCU) and extruded via Na/Ca/Li Exchanger (NCLX). However, the exact contribution of MCU and NCLX in ADP-mediated platelet responses remains incompletely understood.

Daily rhythm in cortical chloride homeostasis underpins functional changes in visual cortex excitability.

Cortical activity patterns are strongly modulated by fast synaptic inhibition mediated through ionotropic, chloride-conducting receptors. Consequently, chloride homeostasis is ideally placed to regulate activity. We therefore investigated the stability of baseline [Cl] in adult mouse neocortex, using in vivo two-photon imaging.

Anti-PF4 immunothrombosis without proximate heparin or adenovirus vector vaccine exposure.

Platelet-activating anti-platelet factor 4 (PF4)/heparin antibodies and anti-PF4 antibodies cause heparin-induced thrombocytopenia (HIT) and vaccine-induced immune thrombocytopenia and thrombosis (VITT), respectively. Diagnostic and treatment considerations differ somewhat between HIT and VITT. We identified patients with thrombocytopenia and thrombosis without proximate heparin exposure or adenovirus-based vaccination who tested strongly positive by PF4/polyanion enzyme-immunoassays and negative/weakly positive by heparin-induced platelet activation (HIPA) test but strongly positive by PF4-induced platelet activation (PIPA) test (ie, VITT-like profile).

[Hematological workup in cases of recurrent early miscarriages: what evidences?].

Recurrent miscarriages have a major psychological and somatic impact, as well as a significant economic burden. An etiological work-up should be offered after two or three miscarriages, the threshold varying from one scientific society to another. However, the proposed biological work-up must be justified by scientific evidence.

Eosinophilic Granulomatosis with Polyangiitis after mRNA-1273 SARS-CoV-2 Vaccine.

During one of the worst global health crises, millions of people were vaccinated against SARS-CoV-2. In rare cases, new onset systemic inflammatory diseases were reported with temporal coincidence to the vaccination. We describe a case of severe Eosinophilic Granulomatosis with Polyangiitis (EGPA) in a young asthmatic woman, occurring after a second dose of the mRNA-1273 vaccine.

Ischemic Stroke in Cancer: Mechanisms, Biomarkers, and Implications for Treatment.

Ischemic stroke is an important cause of morbidity and mortality in cancer patients. The underlying mechanisms linking cancer and stroke are not completely understood. Long-standing and more recent evidence suggests that cancer-associated prothrombotic states, along with treatment-related vascular toxicity, such as with chemotherapy and immunotherapy, contribute to an increased risk of ischemic stroke in cancer patients.

Recommendations for the use of andexanet alfa in the management of bleeding in patients on oral factor Xa inhibitors in Switzerland: Guideline from the Working Party Hemostasis of the Swiss Society of Hematology.

Anticoagulants are essential in preventing and treating thrombosis. Unfortunately, their use is accompanied by an enhanced risk of bleeding. Since the introduction of direct oral anticoagulants (DOACs), the risk of major bleeding has been reduced but not eliminated.

Identification of High Platelet Reactivity Despite ADP P2Y Inhibitor Treatment: Two Populations in the Vasodilator-Stimulated Phosphoprotein Assay and Variable PFA-P2Y Shapes of Curve.

 Response to ADP P2Y receptor inhibition by clopidogrel can be evaluated by various techniques. Here, we compared a functional rapid point-of-care technique (PFA-P2Y) with the degree of biochemical inhibition assessed by the VASP/P2Y assay.  Platelet response to clopidogrel was investigated in 173 patients undergoing elective intracerebral stenting (derivation cohort  = 117; validation cohort  = 56).