Spanish Society of Hematology and Hemotherapy expert consensus opinion for SARS-CoV-2 vaccination in onco-hematological patients.

Abstract In the midst of the COVID-19 pandemic, different vaccines in front of SARS-CoV-2 have been approved and administered in different vulnerable populations. As patients with cancer were excluded from pivotal trials of vaccination, little is known on their immunogenic response to these vaccines, particularly in patients with severely impaired immune system. In response to that uncertainty, the Spanish Society of Hematology and Hemotherapy launched an initiative aimed to provide recommendations for vaccination of the main hematological conditions. This document is based on the available information on COVID-19 outcomes, prior knowledge on vaccination in hematological patients, recent pu…

Electromyographic and Safety Comparisons of Common Lower Limb Rehabilitation Exercises for People With Hemophilia.

Abstract Background Ankles and knees are commonly affected in people with hemophilia and thus are targets for prevention or rehabilitation. However, to our knowledge, no studies have evaluated muscle activity and safety during exercises targeting the lower limbs in people with hemophilia; this lack of information hinders clinical decision-making. Objective The aim of this study was to compare the tolerability of, safety of, and muscle activity levels obtained with external resistance (elastic or machine)-based and non–external resistance-based lower limb exercises in people with hemophilia. Design This was a cross-sectional study. Methods Eleven people who had severe hemophilia and were und…

HemoKinect: A Microsoft Kinect V2 Based Exergaming Software to Supervise Physical Exercise of Patients with Hemophilia

Patients with hemophilia need to strictly follow exercise routines to minimize their risk of suffering bleeding in joints, known as hemarthrosis. This paper introduces and validates a new exergaming software tool called HemoKinect that intends to keep track of exercises using Microsoft Kinect V2&rsquo

Feasibility, safety and muscle activity during flywheel vs traditional strength training in adult patients with severe haemophilia

INTRODUCTION: Eccentric training has been associated with several specific physiological adaptations. The flywheel machine is one of the easiest ways of performing eccentric overload training. However, no studies evaluated its feasibility, safety and muscle activity in patients with haemophilia (PWH).AIM: To evaluate feasibility and safety and compare muscle activity during flywheel vs weight machine knee extension exercise in severe PWH.METHODS: Eleven severe PWH [mean age of 33.5 (8.1) years] participated in this cross-sectional study after receiving prophylactic treatment. Surface electromyography (EMG) signals were recorded for the rectus femoris during the knee extension exercise perfo…

Association of Vitamin K and Non-Vitamin K Oral Anticoagulant Use and Cancer Incidence in Atrial Fibrillation Patients

The association between the use of vitamin K antagonists (VKAs) and cancer risk reduction remains unclear. We aimed to assess the association between the use of VKAs or direct oral anticoagulants (DOACs) and the incidence of cancer in a large cohort of patients with atrial fibrillation (AF) by means of a population-based, propensity-weighted cohort study using population-wide databases including patients diagnosed with nonvalvular AF (NVAF) followed for up of 5 years (median 2.94 years). We created two cohorts based on the initiation therapy (VKA or DOAC). Initiation with VKA or DOAC was defined as filling a prescription with no previous exposure in the preceding 12 months. Cancer diagnoses…

Predictive factors of immune tolerance treatment response in severe haemophilia A patients with inhibitors: A real‐world report from a single centre, mixed retrospective‐prospective long‐term study

Effects of a non-pharmacological approach for chronic pain management in patients with haemophilia: efficacy of cognitive-behavioural therapy associated with physiotherapy.

INTRODUCTION More than half of adult patients with severe haemophilia (PWH) suffer pain daily, with chronic pain (CP) in more than 15% of cases, thereby reducing their quality of life (QoL). However, there are no evidence-based therapeutic guidelines for pain management. AIM To evaluate the effectiveness of a combined protocol based on psychology and physiotherapy in the improvement of CP self-efficacy in PWH with CP. Secondary outcomes are changes in QoL, emotional status, pain and kinesiophobia. METHODS In this prospective controlled trial study, recruited patients were allocated either to an experimental group (EG, n = 10) or to a control group (CG, n = 9). EG received interventions over…

Safety and Effectiveness of Progressive Moderate-to-Vigorous Intensity Elastic Resistance Training on Physical Function and Pain in People With Hemophilia.

Abstract Objective Strength training is recommended for people with hemophilia; however, published data are anecdotal and have methodological limitations. The purpose of this study was to evaluate the safety and effectiveness of progressive moderate-to-vigorous intensity elastic resistance training on physical function and pain in this patient population. Methods A randomized controlled trial was conducted in a university laboratory setting where 20 patients (17 with severe, 1 with moderate, and 2 with mild hemophilia) aged 21 to 53 years received evaluations at baseline and 8-week follow-up. Participants were allocated to intervention (progressive strength training) or control (usual daily…

Home‐delivered ultrasound monitoring for home treatment of haemarthrosis in haemophilia A

Ultrasound evaluation of joint damage and disease activity in adult patients with severe haemophilia A using the HEAD‐US system

Introduction The Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) system and scoring scale has proven to be an accurate and time-efficient imaging method for identifying joint damage in patients with haemophilia. Aim Observational, multicentre, cross-sectional study conducted in 8 centres in Spain that assessed the joint status of adult patients with severe haemophilia A (SHA) using HEAD-US. Methods Joint status of the elbow, knee and ankle was evaluated in adults with SHA receiving on-demand (OD) treatment, or primary (PP), secondary (SP), tertiary (TP) or intermittent (IP) prophylaxis. Results Of the 95 patients enrolled, 87 received prophylaxis (6.3% PP, 38.9% SP, 43.2% …

Using pharmacokinetics for tailoring prophylaxis in people with hemophilia switching between clotting factor products: A scoping review.

Abstract The objective of this scoping review is to summarize the current use of pharmacokinetics for tailoring prophylaxis in hemophilia patients switching between clotting factor products. Patients with hemophilia may require switching of clotting factor concentrates due to a variety of factors, but there have been perceived risks associated with switching, such as inhibitor development or suboptimal protection due to inadequate dosing while titrating treatment. Studies that look at patients switching from one clotting factor concentrate to another are categorized in terms of their primary and/or secondary objectives, notably biosimilarity and comparative pharmacokinetic studies and inhib…

Clinical benefits of a Bayesian model for plasma-derived factor VIII/VWF after one year of pharmacokinetic-guided prophylaxis in severe/moderate hemophilia A patients

Abstract Introduction Individual pharmacokinetic (PK) profiling in hemophilia A (HA) helps to individualize prophylaxis using population PK models (popPK). A specific popPK model for plasma-derived factor VIII containing von-Willebrand Factor (pdFVIII/VWF) was developed. Aim To compare standard versus PK-driven prophylaxis, using a generic or a specific popPK model for pdFVIII/VWF. Materials and methods A prospective study conducted in HA patients in prophylaxis with pdFVIII/VWF (Fanhdi®) comparing three one-year study periods: (1) standard prophylaxis, (2) PK-guided prophylaxis using a generic pdFVIII popPK model which described FVIII activity irrespective of FVIII concentrate, and (3) PK-…

Effect of radiosynoviorthesis on the progression of arthropathy and haemarthrosis reduction in haemophilic patients.

IntroductionRepeated haemarthrosis is widely accepted as the triggering cause of synovitis and haemophilic arthropathy. A first-line treatment of chronic synovitis is radiosynoviorthesis (RS). The aim of this study was to evaluate the RS effects on the progression of arthropathy and on a reduction in bleeding in patients with haemophilia. MethodsAn observational-retrospective study was performed. Bleeding episodes in the 12months following and in the 12months preceding RS was compared. The arthropathy was clinically and radiologically analysed by age range, joint and subject, comparing those undergoing RS (Radiosynoviorthesis Group, RSG) against those not undergoing this treatment (Non-Radi…

Cross‐sectional comparative study of pharmacokinetics and efficacy between sucrose‐formulated recombinant factor VIII (Kogenate ® ) and BAY 81‐8973 (Kovaltry ® ) in patients with severe or moderate haemophilia A in prophylaxis

Physical Activity Monitoring and Acceptance of a Commercial Activity Tracker in Adult Patients with Haemophilia.

Physical activity (PA) is highly beneficial for people with haemophilia (PWH), however, studies that objectively monitor the PA in this population are scarce. This study aimed to monitor the daily PA and analyse its evolution over time in a cohort of PWH using a commercial activity tracker. In addition, this work analyses the relationship between PA levels, demographics, and joint health status, as well as the acceptance and adherence to the activity tracker. Twenty-six PWH were asked to wear a Fitbit Charge HR for 13 weeks. According to the steps/day in the first week, data were divided into two groups: Active Group (AG

Quantification of physical activity in adult patients with haemophilic arthropathy in prophylaxis treatment using a fitness tracker

Bayesian pharmacokinetic-guided prophylaxis with recombinant factor VIII in severe or moderate haemophilia A

Introduction: Personalised pharmacokinetics (PK) using Bayesian analysis with limited sampling is assumed to help to optimise prophylaxis in haemophilia A (HA) patients. Materials and methods: Our prospective, observational study analysed the influence of PK parameters on clinical variables (bleeding rates, joint status, adherence, and consumption) using myPKFiT (R) in a cohort of twenty-one severe and moderate HA patients on prophylaxis with recombinant FVIII (Advate (R)) in two periods of one year, the first before PK-based tailoring and the second after PK-guided prophylaxis. Intra-individual and inter-individual coefficients of variation (CV) of half-life (t(1/2)) were calculated. Resul…

Upper-Body Exercises With External Resistance Are Well Tolerated and Enhance Muscle Activity in People With Hemophilia

Abstract Background Conventional nonresisted therapeutic exercises for people with hemophilia involve a careful, low-intensity approach to avoid injuries. Externally resisted exercise is highly efficient for increasing muscle strength in healthy adults but its feasibility for people with hemophilia remains unknown. Objective The purpose of this study was to evaluate muscle activity during upper-body rehabilitation exercises with 2 types of external resistance and without external resistance (conventional) and to examine tolerability, kinesiophobia, and possible adverse effects derived from the session. Design This was a cross-sectional study. Methods Twelve people with hemophilia A/B (11 wi…

Assessment of Kinect V2 for elbow range of motion estimation in people with haemophilia using an angle correction model

Introduction The joint range of motion (ROM) is an important clinical parameter used to assess the loss of functionality resulting from joint bleedings in people with haemophilia. These episodes require a close follow-up and, to decrease patients' hospital dependence, telemedicine tools are needed. Therefore, this study is aimed to analyse the validity of the Microsoft Kinect V2 sensor with corrected angle measurement to be used in the monitoring of elbow ROM in people with haemophilia. Methods A convenience sample of 10 healthy controls (CG) and 10 patients with haemophilia with elbow arthropathy (HG) participated in this study. Full ROM of elbow joints was measured in the frontal view wit…