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.

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 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.

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.

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.

Congenital and acquired bleeding disorders in infancy.

The diagnosis of congenital and acquired bleeding disorders in infants requires an understanding of developmental haemostasis and the effect on laboratory testing. A systematic approach to bleeding in neonates will aid clinicians in the diagnosis and treatment, which may be caused by a wide variety of diseases. The clinical setting will help to direct the diagnostic pathway.

Extreme warfarin hypersensitivity after oophorectomy.

We report the case of a woman who developed unexplained warfarin hypersensitivity after undergoing surgery to remove her ovaries. Presurgery, the patient's international normalised ratios (INR) control was stable and uneventful but 11 days after her operation she presented with extremely high (frequently ≥10) INR. Warfarin was discontinued on day 24 postoperation but 11 days later the plasma warfarin concentration was high at 4.

Sample conditions determine the ability of thrombin generation parameters to identify bleeding phenotype in FXI deficiency.

Individuals with Factor XI (FXI) deficiency have a variable bleeding tendency that does not correlate with FXI:C levels or genotype. Comparing a range of sample conditions, we tested whether the thrombin generation assay (TGA) could discriminate between control subjects (n = 50) and FXI-deficient individuals (n = 97), and between those with bleeding tendency (n = 50) and without (n = 24). The comparison used platelet-rich plasma (PRP) and platelet-poor plasma (PPP), either with or without corn trypsin inhibitor (CTI) to prevent contact activation, over a range of tissue factor (TF) concentrations.

Wrong blood in tube - potential for serious outcomes: can it be prevented?

'Wrong blood in tube' (WBIT) errors, where the blood in the tube is not that of the patient identified on the label, may lead to catastrophic outcomes, such as death from ABO-incompatible red cell transfusion. Transfusion is a multistep, multidisciplinary process in which the human error rate has remained unchanged despite multiple interventions (education, training, competency testing and guidelines). The most effective interventions are probably the introduction of end-to-end electronic systems and a group-check sample for patients about to receive their first transfusion, but neither of these eradicates all errors.

Transfusion and hemovigilance in pediatrics.

Hemovigilance is an essential part of the transfusion process and is defined as surveillance procedures covering the whole transfusion chain, from collection of blood and its components, intended to collect and assess information on unexpected or undesirable effects resulting from the therapeutic use of labile blood products and to prevent their occurrence or recurrence. The UK surveillance scheme has collected data for 16 years and is a model demonstrating how information on adverse incidents can be used to improve patient safety, influencing the management of donors and improved education and training for the many people involved in the transfusion process.

Serious Hazards of Transfusion (SHOT) haemovigilance and progress is improving transfusion safety.

The Serious Hazards of Transfusion (SHOT) UK confidential haemovigilance reporting scheme began in 1996. Over the 16 years of reporting, the evidence gathered has prompted changes in transfusion practice from the selection and management of donors to changes in hospital practice, particularly better education and training. However, half or more reports relate to errors in the transfusion process despite the introduction of several measures to improve practice.

Commentary on session: Immune thrombocytopenia nomenclature, guidelines, and natural history.

Two presentations discussed different aspects of immune thrombocytopenia (ITP) management. The first considered active monitoring for occult hemorrhage in the gastrointestinal tract, urinary tract, and brain. Participants generally did not feel that these would be useful in determining management of children with ITP since serious bleeding was likely to manifest itself.

Bleeding manifestations and management of children with persistent and chronic immune thrombocytopenia: data from the Intercontinental Cooperative ITP Study Group (ICIS).

Long-term follow-up of children with immune thrombocytopenia (ITP) indicates that the majority undergo remission and severe thrombocytopenia is infrequent. Details regarding bleeding manifestations, however, remain poorly categorized. We report here long-term data from the Intercontinental Cooperative ITP Study Group Registry II focusing on natural history, bleeding manifestations, and management.

A genome-wide association study of resistance to HIV infection in highly exposed uninfected individuals with hemophilia A.

Human genetic variation contributes to differences in susceptibility to HIV-1 infection. To search for novel host resistance factors, we performed a genome-wide association study (GWAS) in hemophilia patients highly exposed to potentially contaminated factor VIII infusions. Individuals with hemophilia A and a documented history of factor VIII infusions before the introduction of viral inactivation procedures (1979-1984) were recruited from 36 hemophilia treatment centers (HTCs), and their genome-wide genetic variants were compared with those from matched HIV-infected individuals.

The rare inherited coagulation disorders.

The rare inherited coagulation disorders (RICD) are uncommon and thus not well-defined in terms of severity or management. Inheritance is autosomal; in some of these disorders in the heterozygote state affected individuals may be mildly symptomatic. Severe deficiencies are more common in association with consanguinity.

Rare bleeding disorders.

Rare bleeding disorders (RBDs) include the inherited deficiencies of fibrinogen, factor (F)II, FV, FV+FVIII, FVII, FX, FXI and FXIII. There have been remarkable advances in understanding the molecular profiles that lead to each type of coagulation factor deficiency. However, as a consequence of their rarity, clinical data regarding the characteristics of bleeding symptoms and their management remain limited.