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View article: Appropriate Management of Thrombotic Risk in Patients With Primary Immune Thrombocytopenia in the UK: A Modified Delphi Consensus
Appropriate Management of Thrombotic Risk in Patients With Primary Immune Thrombocytopenia in the UK: A Modified Delphi Consensus Open
Introduction Immune thrombocytopenia (ITP) is characterised by a low platelet count and increased risk of bleeding. Recent research has also proposed that having ITP increases thrombosis risk. Moreover, certain ITP treatments have been ass…
View article: Laboratory practice on detection of antiphospholipid antibodies: UK NEQAS blood coagulation survey—2024
Laboratory practice on detection of antiphospholipid antibodies: UK NEQAS blood coagulation survey—2024 Open
View article: Recombinant Adeno-Associated Virus Integration Profiles in Nonhuman Primates and Gene Therapy Participants after Treatment with Valoctocogene Roxaparvovec
Recombinant Adeno-Associated Virus Integration Profiles in Nonhuman Primates and Gene Therapy Participants after Treatment with Valoctocogene Roxaparvovec Open
Recombinant adeno-associated viruses (AAVs) are clinically relevant vectors for gene therapy that persist largely as extrachromosomal episomes but also infrequently integrate into host genomes. Valoctocogene roxaparvovec is an approved AAV…
View article: Anti‐<scp>PF4</scp> mediated thrombocytopenia and thrombosis associated with acute cytomegalovirus infection displays both <scp>HIT</scp>‐like and <scp>VITT</scp>‐like characteristics
Anti‐<span>PF4</span> mediated thrombocytopenia and thrombosis associated with acute cytomegalovirus infection displays both <span>HIT</span>‐like and <span>VITT</span>‐like characteristics Open
Summary Vaccine‐induced immune thrombocytopenia and thrombosis (VITT) is one of several anti‐platelet factor 4 (anti‐PF4)‐associated immune thrombocytopenia and thrombosis (PITT) syndromes. As well as following adenoviral vector vaccines, …
View article: Alternative Immunosuppression in Acquired Haemophilia A
Alternative Immunosuppression in Acquired Haemophilia A Open
View article: The diagnostic utility of genetic testing in inherited thrombocytopenia: regional multicenter tertiary experience
The diagnostic utility of genetic testing in inherited thrombocytopenia: regional multicenter tertiary experience Open
View article: Demystifying autoimmune HIT: what it is, when to test, and how to treat
Demystifying autoimmune HIT: what it is, when to test, and how to treat Open
Antibodies to platelet factor 4 (PF4) have been primarily linked to classical heparin-induced thrombocytopenia (cHIT). However, during the rollout of the COVID-19 vaccine program a new condition, vaccine-induced thrombocytopenia and thromb…
View article: Valoctocogene roxaparvovec gene therapy provides durable haemostatic control for up to 7 years for haemophilia A
Valoctocogene roxaparvovec gene therapy provides durable haemostatic control for up to 7 years for haemophilia A Open
Introduction Valoctocogene roxaparvovec is an adeno‐associated virus vector serotype 5 (AAV5)‐mediated gene therapy approved for severe haemophilia A (HA). Aim To report the safety and efficacy of valoctocogene roxaparvovec 7 years after d…
View article: The safety and efficacy of using age-adjusted D-dimers in hospitalised patients in a diverse urban centre: a real-world data study
The safety and efficacy of using age-adjusted D-dimers in hospitalised patients in a diverse urban centre: a real-world data study Open
Objective Despite recommendations, age adjusted thresholds (AAT) for D Dimers are not routinely used as part of venous thromboembolism (VTE) screening in many healthcare settings due to concerns about missing cases, especially in older and…
View article: Guideline for laboratory diagnosis and monitoring of von Willebrand disease: A joint guideline from the United Kingdom Haemophilia Centre Doctors' Organisation and the British Society for Haematology
Guideline for laboratory diagnosis and monitoring of von Willebrand disease: A joint guideline from the United Kingdom Haemophilia Centre Doctors' Organisation and the British Society for Haematology Open
This guideline updates the previous guidelines1, 2 published on behalf of the British Society for Haematology (BSH) and the United Kingdom Haemophilia Centre Doctors' Organisation (UKHCDO), focussing on the laboratory components of diagnos…
View article: A British Society for Haematology guideline on the assessment and management of bleeding risk prior to invasive procedures
A British Society for Haematology guideline on the assessment and management of bleeding risk prior to invasive procedures Open
This guideline was compiled according to the British Society for Haematology (BSH) process at https://b-s-h.org.uk/. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) nomenclature was used to evaluate the levels…
View article: Long‐term safety and efficacy outcomes of valoctocogene roxaparvovec gene transfer up to 6 years post‐treatment
Long‐term safety and efficacy outcomes of valoctocogene roxaparvovec gene transfer up to 6 years post‐treatment Open
Introduction Valoctocogene roxaparvovec uses an adeno‐associated virus serotype 5 (AAV5) vector to transfer a factor VIII (FVIII) coding sequence to individuals with severe haemophilia A, providing bleeding protection. Aim To assess safety…
View article: The Safety and Efficacy an Age-Adjusted D-Dimer Threshold in Hospitalised Patients with Suspected Venous Thromboembolic Events in a Diverse Urban Centre: A Real-World Data Study
The Safety and Efficacy an Age-Adjusted D-Dimer Threshold in Hospitalised Patients with Suspected Venous Thromboembolic Events in a Diverse Urban Centre: A Real-World Data Study Open
View article: Interest and barriers to research in obstetric haematology – findings from a national survey in the United Kingdom
Interest and barriers to research in obstetric haematology – findings from a national survey in the United Kingdom Open
Introduction In 2021, the steering committee members of British Society of Haematology Obstetric Haematology Special Interest Group noted difficulties in opening research studies. This led to the development of a survey to further evaluate…
View article: Immune thrombotic thrombocytopenic purpura in patients over 60 years of age: Diagnostic pitfalls and treatment strategy
Immune thrombotic thrombocytopenic purpura in patients over 60 years of age: Diagnostic pitfalls and treatment strategy Open
Real‐world registry studies of older patients with iTTP highlight diagnostic difficulties in comparison to patients less than 60 years of age with greater risk of renal injury, atypical neurological features and less profound cytopenia, wh…
View article: A British Society for Haematology Guideline: Diagnosis and management of thrombotic thrombocytopenic purpura and thrombotic microangiopathies
A British Society for Haematology Guideline: Diagnosis and management of thrombotic thrombocytopenic purpura and thrombotic microangiopathies Open
Summary The objective of this guideline is to provide healthcare professionals with clear, up‐to‐date and practical guidance on the management of thrombotic thrombocytopenic purpura (TTP) and related thrombotic microangiopathies (TMAs), in…
View article: Reassurance on bleeding and thrombotic events following second dose BNT162b2 and ChAdOx1 COVID-19 vaccines
Reassurance on bleeding and thrombotic events following second dose BNT162b2 and ChAdOx1 COVID-19 vaccines Open
View article: British Society for Haematology guideline for anticoagulant management of pregnant individuals with mechanical heart valves
British Society for Haematology guideline for anticoagulant management of pregnant individuals with mechanical heart valves Open
Evidence is limited regarding the prevalence and optimal management of pregnancy in individuals with mechanical heart valves (MHVs). Studies are scarce, often with small numbers of patients included. Mechanical valve thrombosis (MVT) occur…
View article: Effectiveness of clinical decision support in controlling inappropriate red blood cell and platelet transfusions, speciality specific responses and behavioural change
Effectiveness of clinical decision support in controlling inappropriate red blood cell and platelet transfusions, speciality specific responses and behavioural change Open
Background Electronic clinical decision support (CDS) within Electronic Health Records has been used to improve patient safety, including reducing unnecessary blood product transfusions. We assessed the effectiveness of CDS in controlling …
View article: Long-term risk of relapse in immune-mediated thrombotic thrombocytopenic purpura and the role of anti-CD20 therapy
Long-term risk of relapse in immune-mediated thrombotic thrombocytopenic purpura and the role of anti-CD20 therapy Open
Disease relapse is recognized as a risk in immune-mediated thrombotic thrombocytopenic purpura (iTTP) after treatment of the acute presenting episode. Identification of patients at risk of relapse and its patterns are yet to be clearly est…
View article: Missense mutations in <i>PIEZO1,</i> which encodes the Piezo1 mechanosensor protein, define Er red blood cell antigens
Missense mutations in <i>PIEZO1,</i> which encodes the Piezo1 mechanosensor protein, define Er red blood cell antigens Open
Despite the identification of the high-incidence red cell antigen Era nearly 40 years ago, the molecular background of this antigen, together with the other 2 members of the Er blood group collection, has yet to be elucidated. Whole exome …
View article: Systematic approach to outcome assessment from coded electronic healthcare records in the DaRe2THINK NHS-embedded randomized trial
Systematic approach to outcome assessment from coded electronic healthcare records in the DaRe2THINK NHS-embedded randomized trial Open
Aims Improving the efficiency of clinical trials is key to their continued importance in directing evidence-based patient care. Digital innovations, in particular the use of electronic healthcare records (EHRs), allow for large-scale scree…
View article: Unmet needs in the management of immune‐mediated thrombotic thrombocytopenic purpura and the potential role of caplacizumab in the UK—A modified‐Delphi study
Unmet needs in the management of immune‐mediated thrombotic thrombocytopenic purpura and the potential role of caplacizumab in the UK—A modified‐Delphi study Open
Immune‐mediated thrombotic thrombocytopenic purpura (iTTP) is an ultra‐rare, blood‐clotting disorder. Management historically relies on plasma exchange and immunosuppression; however, a 10%–20% mortality rate is still observed. Caplacizuma…
View article: Interindividual variability in transgene mRNA and protein production following adeno-associated virus gene therapy for hemophilia A
Interindividual variability in transgene mRNA and protein production following adeno-associated virus gene therapy for hemophilia A Open
View article: Natural history of PF4 antibodies in vaccine-induced immune thrombocytopenia and thrombosis
Natural history of PF4 antibodies in vaccine-induced immune thrombocytopenia and thrombosis Open
The COVID-19 pandemic has resulted in the rapid development of a range of vaccines against SARS-CoV-2. Vaccine-induced immune thrombocytopenia and thrombosis (VITT) is a rare but life-threatening complication of primarily adenoviral-based …
View article: Anti‐platelet factor 4 immunoglobulin G levels in vaccine‐induced immune thrombocytopenia and thrombosis: Persistent positivity through 7 months
Anti‐platelet factor 4 immunoglobulin G levels in vaccine‐induced immune thrombocytopenia and thrombosis: Persistent positivity through 7 months Open
Explanations for reduced platelet activation during follow-up, despite similar total anti-PF4 antibody levels, remains unclear. Clinical implications of persistent anti-PF4 antibodies in VITT require further study.
View article: Acquired thrombotic thrombocytopenic purpura: A rare disease associated with BNT162b2 vaccine: Comment from Doyle et al.
Acquired thrombotic thrombocytopenic purpura: A rare disease associated with BNT162b2 vaccine: Comment from Doyle et al. Open
View article: Diagnostic uncertainty presented barriers to the timely management of acute thrombotic thrombocytopenic purpura in the United Kingdom between 2014 and 2019
Diagnostic uncertainty presented barriers to the timely management of acute thrombotic thrombocytopenic purpura in the United Kingdom between 2014 and 2019 Open
View article: Rare missense variants in Tropomyosin‐4 (TPM4) are associated with platelet dysfunction, cytoskeletal defects, and excessive bleeding
Rare missense variants in Tropomyosin‐4 (TPM4) are associated with platelet dysfunction, cytoskeletal defects, and excessive bleeding Open
Genetic and functional TPM4 defects are reported making TPM4 a diagnostic grade tier 1 gene and highlights the importance of including TPM4 in diagnostic genetic screening for patients with significant bleeding and undiagnosed platelet dis…
View article: Just how common is TTS after a second dose of the ChAdOx1 nCov-19 vaccine?
Just how common is TTS after a second dose of the ChAdOx1 nCov-19 vaccine? Open