Intensive Therapeutic Plasma Exchange-New Approach to Treat and Rescue Patients with Severe Form of Yellow Fever.

Background: Severe yellow fever (YF) can result in acute liver failure (ALF) and high mortality. The role of therapeutic plasma exchange (TPE) in managing YF-ALF remains unclear. This study evaluated the impact of TPE strategies in severe YF.

Safety and efficacy of valoctocogene roxaparvovec with prophylactic glucocorticoids: 1-year results from the phase 3b, single-arm, open-label GENEr8-3 study.

Background: Valoctocogene roxaparvovec, an adeno-associated virus vector that transfers a human factor (F)VIII (FVIII) coding sequence to hepatocytes, provides bleeding protection for people with severe hemophilia A.

Objectives: Determine the efficacy and safety of valoctocogene roxaparvovec with concomitant prophylactic glucocorticoids in the open-label, single-arm, phase 3b GENEr8-3 trial.

Methods: Participants with severe hemophilia A who were using hemophilia A prophylaxis received one 6 × 10 vg/kg infusion of valoctocogene roxaparvovec concomitantly with daily prophylactic glucocorticoids (40 mg prednisolone equivalent/d weeks 0-8; taper to 5 mg/d weeks 9-19).

Persistent hypofibrinolysis in severe COVID-19 associated with elevated fibrinolysis inhibitors activity.

Hypercoagulability and reduced fibrinolysis are well-established complications associated with COVID-19. However, the timelines for the onset and resolution of these complications remain unclear. The aim of this study was to evaluate, in a cohort of COVID-19 patients, changes in coagulation and fibrinolytic activity through ROTEM assay at different time points during the initial 30 days following the onset of symptoms in both mild and severe cases.

Extracellular vesicles are a late marker of inflammation, hypercoagulability and COVID-19 severity.

Exacerbated inflammation and coagulation are a hallmark of COVID-19 severity. Extracellular vesicles (EVs) are intercellular transmitters involved in inflammatory conditions, which are capable of triggering prothrombotic mechanisms. Since the release of EVs is potentially associated with COVID-19-induced coagulopathy, the aim of this study was to evaluate changes in inflammation- and hypercoagulability-related EVs during the first month after symptom onset and to determine whether they are associated with disease severity.

Extended half-life recombinant factor VIII treatment of hemophilia A in Brazil: an expert consensus statement.

Introduction: Treatment of hemophilia A in Brazil is offered to all patients at no cost. However, several unmet medical needs exist.

Method: In this study, we applied the Delphi method to discuss with seven hemophilia A specialists the challenges that patients and the health system face regarding hemophilia A treatment and opportunities for improvement.

Detection of alloimmunization in Glanzmann Thrombasthenia and Bernard-Soulier Syndrome: Data from a Brazilian Center.

Introduction: The Glanzmann Thrombasthenia (GT) and Bernard-Soulier Syndrome (BSS) are rare hereditary disorders of platelet function. Their treatment often requires platelet transfusion, which can lead to the development of alloantibodies.

Objective: In this study, we aim to develop a strategy for alloantibody detection and to describe the frequency of alloimmunization in a patient population from a single center in southeastern Brazil.

Immunogenicity, long term protection and safety of subcutaneous administration of hepatitis A vaccine in patients with hemophilia and other bleeding disorders: A randomized study.

Hepatitis A vaccine is recommended for all individuals with hemophilia, although patients with bleeding disorders should avoid intramuscular (IM) injections. To date, only few studies showed subcutaneous (SC) route immunogenicity is comparable with the IM route. Therefore, this randomized study compared immunogenicity, long term protection and safety of hepatitis A vaccine administered by SC route with the IM route in 78 children and adults with hemophilia and other bleeding disorders.

Acquired hemophilia A in a patient with advanced prostate cancer.

Acquired hemophilia A (AHA) is a rare disorder that results from the presence of autoantibodies against the clotting factor VIII (FVIII) causing hemorrhagic disorders. This entity is mostly associated with autoimmune diseases, pregnancy, the postpartum period, drugs and malignancy. Among the solid cancers, prostate neoplasm is the most common cause of AHA.

Subjective results of joint lavage and viscosupplementation in hemophilic arthropathy.

Objective: To assess whether joint lavage, viscosupplementation and triamcinolone improve joint pain, function and quality of life in patients with severe hemophilic arthropathy.

Methods: Fourteen patients with knee and/or ankle hemophilic arthritis with and without involvement of other joints underwent joint lavage and subsequent injection of hylan G-F20 and triamcinolone in all affected joints. The patients answered algo-functional questionnaires (Lequesne and WOMAC), visual analog scale for pain (VAS) and SF-36 preoperatively, and at one, three, six and twelve months postoperatively.

Arthroscopic partial anterior synovectomy of the knee on patients with haemophilia.

Purpose: This study assessed the results of two-portal knee arthroscopic synovectomy in terms of bleeding recurrence, knee function, quality of life (QOL), and radiographic staging in a prospective case series of patients with haemophilia.

Methods: Nine knees from eight patients (median age 16.1 years; range 9.

Diagnosis and treatment of congenital hemophilia with inhibitors a Latin American perspective.

The Committee of Latin America on the Therapeutics of Inhibitor Groups (CLOTTING) is composed of a number of hemophilia specialists from Latin America. The group aims to encourage the adoption of a good standard of care for Latin American patients with hemophilia. The occurrence of inhibitors in patients with hemophilia poses clinical challenges, and it is estimated that between 1000 and 3000 patients in Latin America are affected by hemophilia with inhibitors.

Ethnic differences in cerebral venous thrombosis.

Background: Cerebral venous thromboses (CVT) with distinct clinical presentations have been shown worldwide. However, there is little information regarding race-ethnic differences in this disease.

Methods: We prospectively studied 50 CVT patients from Brazil, comparing clinical and laboratory data among white (W) and African-Brazilian (AB) patients.