Prevention and treatment of infection in patients with an absent or hypofunctional spleen: A British Society for Haematology guideline.

Guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen were published by the British Committee for Standards in Haematology in 1996 and updated in 2002 and 2011. With advances in vaccinations and changes in patterns of infection, the guidelines required updating. Key aspects included in this guideline are the identification of patients at risk of infection, patient education and information and immunisation schedules.

Tissue factor pathway inhibitor is a potential modifier of bleeding risk in factor XI deficiency.

Background: Factor (F) XI deficiency is associated with increased bleeding risk in some individuals. Neither FXI levels nor clinical clotting assays predict the bleeding risk. Compared with controls, FXI-deficient bleeders have reduced clot formation, decreased fibrin network density, and increased susceptibility to fibrinolysis.

Transfusion-related Acute Lung Injury: 36 Years of Progress (1985-2021).

The term transfusion-related acute lung injury (TRALI) was coined in 1985 to describe acute respiratory distress syndrome (ARDS) after transfusion, when another ARDS risk factor was absent; TRALI cases were mostly associated with donor leukocyte antibody. In 2001, plasma from multiparous donors was implicated in TRALI in a randomized controlled trial in Sweden. In 2003 and in many years thereafter, the U.

Missed irradiation of cellular blood components for vulnerable patients: Insights from 10 years of SHOT data.

Background: Irradiation of cellular blood components is recommended for patients at risk of transfusion-associated graft-vs-host disease (TA-GvHD). Prestorage leucodepletion (LD) of blood components is standard in the UK since 1999.

Study Design And Methods: Analysis of 10 years' reports from UK national hemovigilance scheme, Serious Hazards of Transfusion (2010-2019), where patients failed to receive irradiated components when indicated according to British Society for Haematology guidelines (2011).

The haemoglobinopathy survey: The reality of transfusion practice in sickle cell disease and thalassaemia in England.

Objectives: To establish, in an unselected population of London haemoglobinopathy patients, transfusion requirements, blood antigens/alloantibodies, transfusion modalities, burden of transfusion reactions and donor exposure.

Background: Haemoglobinopathy patients are among the most highly transfused patient populations, and the overall population and number of patients on long-term transfusion programmes are increasing. To provide a safe and efficacious transfusion service for patients, it is important to understand current practice, morbidity associated with transfusion, efficacy of different transfusion modalities and geno-/phenotype requirements.

Pulmonary complications of transfusion: Changes, challenges, and future directions.

The pulmonary complications of transfusion (TACO, TRALI and TAD) are the leading cause of transfusion-related mortality and major morbidity. Advance in this area is essential in improving transfusion safety. This review describes the drivers for change in haemovigilance practice, the influence of recent key publications and future directions.

The World Federation of Hemophilia Annual Global Survey 1999-2018.

Introduction: The World Federation of Hemophilia (WFH) strives to achieve care for all patients with inherited bleeding disorders through research, advocacy, capacity building and education. The WFH developed and implemented the Annual Global Survey (AGS), through which comprehensive demographic and treatment data on bleeding disorders are collected each year from its constituent non-governmental national organizations.

Aim: To describe the development, methodology and achievements of the WFH AGS over the past 20 years.

National comparative audit of blood transfusion: 2014 audit of transfusion services and practice in children and adults with sickle cell disease.

Objectives: To determine the organisational resources in place; what blood was being transfused, why, how, where, when and by whom; whether laboratory support and policies met standards for patients with sickle cell disease (SCD).

Background: SCD affects 14 000 people in the United Kingdom (UK). Standards and guidelines do not cover all aspects of transfusion in SCD and there are no data on their use; people may become very sick without warning presenting to non-specialist hospitals; blood services are increasingly supplying units for transfusion in SCD with little data on their use.

Transfusion errors - can they be eliminated?

The Serious Hazards of Transfusion haemovigilance scheme has documented adverse transfusion incidents for 22 years. Transmission of infection (three in 2018), transfusion-related lung injury (one in 2018) and transfusion-associated graft-versus-host disease (none since 2012) are rare. Despite national recommendations, guidelines and protocols, most incidents more than 85% of incidents are still due to errors in the transfusion process.

Electronic identification systems reduce the number of wrong components transfused.

Background: Errors in hospital transfusion may cause wrong (blood) components to be transfused. This study assessed the value of electronic identification systems (EISs) in reducing wrong component transfusions (WCTs).

Methods: UK hospitals reporting to Serious Hazards of Transfusion were invited to complete an electronic survey about transfusion including the use of EISs.

Revised international surveillance case definition of transfusion-associated circulatory overload: a classification agreement validation study.

Background: Transfusion-associated circulatory overload (TACO) is a major cause of transfusion-related morbidity and mortality in countries with well developed transfusion services. The International Society of Blood Transfusion, the International Haemovigilance Network, and AABB (formerly American Association of Blood Banks), have developed and validated a revised definition of TACO.

Methods: International Haemovigilance Network-member haemovigilance systems (Australia, Austria, Denmark, Finland, Greece, India, Ireland, Italy, Japan, Malta, Netherlands, New Zealand, Norway, Slovenia, United Kingdom and United States) provided cases of respiratory complications categorised by their systems, including clinical parameters listed in the 2017 draft definition (part 1).

A consensus redefinition of transfusion-related acute lung injury.

Background: Transfusion-related acute lung injury (TRALI) is a serious complication of blood transfusion and is among the leading causes of transfusion-related morbidity and mortality in most developed countries. In the past decade, the pathophysiology of this potentially life-threatening syndrome has been increasingly elucidated, large cohort studies have identified associated patient conditions and transfusion risk factors, and preventive strategies have been successfully implemented. These new insights provide a rationale for updating the 2004 consensus definition of TRALI.

Abnormal plasma clot formation and fibrinolysis reveal bleeding tendency in patients with partial factor XI deficiency.

Individuals with factor XI (FXI) deficiency have a variable bleeding risk that cannot be predicted from plasma FXI antigen or activity. This limitation can result in under- or overtreatment of patients and risk of bleeding or thrombosis. Previously, plasma clot fibrinolysis assays showed sensitivity to bleeding tendency in a small cohort of patients with severe FXI deficiency.