A British Society for Haematology Guideline: Diagnosis and management of thrombotic thrombocytopenic purpura and thrombotic microangiopathies.

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), including complement-mediated haemolytic uraemic syndrome (CM HUS); these are defined by thrombocytopenia, microangiopathic haemolytic anaemia (MAHA) and small vessel thrombosis. Within England, all TTP cases should be managed within designated regional centres as per NHSE commissioning for highly specialised services.

Characterisation and comparison of immune response mechanisms in an indigenous and a commercial pig breed after classical swine fever vaccination.

The live attenuated classical swine fever (CSF) vaccine has been successfully used to prevent and control CSF outbreaks for 6 decades. However, the immune response mechanisms against the vaccine remain poorly understood. Moreover, very few reports exist regarding the breed differences in the response to CSF vaccine. In this study, we generated the peripheral blood mononuclear cell transcriptomes of indigenous Ghurrah and commercial Landrace pig breeds, before and 7 days after CSF vaccination. Subsequently, between and within-breed differential gene expression analyses were carried out. Results revealed large differences in pre-vaccination peripheral blood mononuclear cell transcriptome profiles of the two breeds, which were homogenised 7 days after vaccination. Before vaccination, gene set enrichment analysis showed that pathways related to antigen sensing and innate immune response were enriched in Ghurrah, while pathways related to adaptive immunity were enriched in Landrace. Ghurrah exhibited greater immunomodulation compared to Landrace following the vaccination. In Ghurrah, cell-cycle processes and T-cell response pathways were upregulated after vaccination. However, no pathways were upregulated in Landrace after vaccination. Pathways related to inflammation were downregulated in both the breeds after vaccination. Key regulators of inflammation such as IL1A, IL1B, NFKBIA and TNF genes were strongly downregulated in both the breeds after vaccination. Overall, our results have elucidated the mechanisms of host immune response against CSF vaccination in two distinct breeds and revealed common key genes instrumental in the global immune response to the vaccine.

Electronic Prompts Can Reduce The Number Of Unnecessary Clotting Screens Ordered For Patients.

In an attempt to reduce the number of inappropriate clotting screens being performed in our Trust, an electronic prompt was introduced to our haematology requesting system. Over the six month period after introduction of this prompt the number of clotting screen requests reduced by 7001, representing a 21% reduction when compared to the same 6 month period one year earlier. This represented a cost saving of over £98,000 without any increase in adverse incidents being reported related to bleeding complications.

Application of UKHCDO 2004 guidelines in type 1 von Willebrand Disease--a single centre paediatric experience of the implications of altered or removed diagnosis.

Diagnosis of type I von Willebrand Disease (VWD) can be challenging. In 2004, the United Kingdom Haemophilia Centre Doctors' Organisation (UKHCDO) proposed more stringent diagnostic criteria to replace the 1995 guidelines. To determine the true number of cases of type 1 VWD in a single paediatric centre, the 2004 UKHCDO Guideline for the diagnosis of VWD was used to evaluate 114 patients on our type 1 VWD register. Clinical and laboratory data were collected and analysed to see whether they met the criteria for type 1 VWD. Only 8% remained on the type 1 VWD register. 18% have been classified as 'possible type 1 VWD'. Twenty five surgical procedures have since been performed on patients from the group in which the diagnosis was removed without any haemostatic support or bleeding complications. Reaction to the removal of the VWD diagnosis or delivery of an alternative diagnosis was positive for most patients and families. This study is the first to assess the impact of the 2004 UKHCDO Guidelines on the diagnosis of VWD. It provides evidence that the prevalence of type 1 VWD may actually be closer to that of haemophilia instead of the previously reported 1-3% of the general population. We propose that all centres should review their patients with a diagnosis of VWD to revalidate this disease that claims to be our most common inherited bleeding disorder.

Multiple centre evaluation study of ADAMTS13 activity and inhibitor assays.

INTRODUCTION: Accurate evaluation of ADAMTS13 activity is required for the diagnosis and clinical management of thrombotic microangiopathies, and commercial kits are available for routine laboratory use. METHODS: Our study compares the results from Technoclone (Technoclone GmbH, Austria) activity and Inhibitor kits with specialist laboratory reference methods (FRETS and ELISA IgG) and the impact of transporting frozen samples and comparison of results. RESULTS: This multicentre study identified differences in Technoclone activity results compared to specialist testing, which could potentially impact diagnosis. A change in the commercial kit during the study period appears to have rectified the detection levels. Frozen samples provided comparable results between sites. CONCLUSION: With close attention to normal ranges, commercial kits are suitable for use in the clinical diagnosis of thrombotic microangiopathies and frozen transportation of samples between sites is a suitable approach. However, a robust external quality control system is essential to provide an independent evaluation of changes in kit production.

Ultrasonic assessment of uterine emptying in first-trimester abortions induced by intravaginal 15-methyl prostaglandin F2 alpha methyl ester.

Early first-trimester abortions were induced by a single intravaginal pessary containing 3 mg of 15 methyl prostaglandin F2 alpha methyl ester in 17 patients. The procedure was successful in 16 patients (94%). Intrauterine events were observed by means of serial ultrasonic B scans. Pain, bleeding, and dislodgement of the gestational sac had commenced within 6 hours. Significant gastrointestinal side effects occurred in the majority of patients despite medication but they were usually of short duration. Vaginal bleeding, which was not heavy, continued for about 10 days and during most of this time the uterus contained products of conception. It is concluded that the method is effective during the first 7 weeks of gestation.

The routine use of oral prostaglandin E2 tablets for induction or augmentation of labour.

The use of oral prostaglandin E2 (PGE2) tablets is an effective and safe adjunct to low amniotomy for the induction of labour at term. Routine use of this regimen and the use of oral PGE2 tablets for augmentation of spontaneous labour has shown that the method is very effective in multiparous patients, but much less so in primiparous patients. Success or failure could not be predicted by the pre-induction Bishop score. The incidence of gastro-intestinal side effect is dose dependent, but is low in multiparous patients.