Antiphospholipid-related chorea

Chorea can be associated with autoimmune diseases such as antiphospholipid syndrome and has been associated with the isolated presence of antiphospholipid antibodies (aPL). Chorea is a rare neurological manifestation of antiphospholipid syndrome. The pathophysiological mechanisms underlying aPL-related chorea are still debated. One postulated mechanism is aPL or other autoantibody binding to brain-blood vessel endothelium, resulting in endothelial dysfunction secondary to a proinflammatory cascade, with sequalae of inflammation and local microthrombosis. Another postulated mechanism considers immune-mediated attack (aPL or antibasal ganglia antibodies) against specific basal ganglia epitopes. Here, we report a patient with isolated aPL-related chorea that followed a relapsing-remitting course. We highlight the role of brain metabolic imaging with fluorodeoxy glucose positron-emission tomography in the diagnostic workup of chorea and the challenges in the practical management of aPL-related chorea with symptomatic treatments

A call to action: MTHFR polymorphisms should not be a part of inherited thrombophilia testing

Testing for polymorphisms in the methylenetetrahydrofolate reductase (MTHFR) gene is still a standard part of thrombophilia testing in many laboratories. However, it is clear that these polymorphisms are not risk factors for arterial or venous thrombosis and therefore should not be part of thrombophilia testing. Eliminating MTHFR from thrombophilia testing will reduce patient concerns and health care costs

VITT, COVID-19 and the Expert Haematology Panel: the story of how the UK responded to emerging cases of vaccine-induced immune thrombocytopenia and thrombosis during the vaccination programme

The COVID-19 pandemic has resulted in the development of highly effective vaccines that provide hope to the global community for reducing the spread of SARS-CoV-2 and limiting the mortality and morbidity caused by the disease. These vaccines have been produced using differing technologies, taken through clinical trials, and rolled out across the UK at unprecedented speed. However, the recent emergence of rare cases of life-threatening thrombosis in association with thrombocytopenia has threatened to derail one particular vaccine, the Oxford AstraZeneca ChAdOx1 vaccine, upon which many countries are dependent for their vaccination programmes. The story of how this situation has been managed in the UK at the height of the vaccine roll-out represents a remarkable collective endeavour on the part of the haematology community, working closely with other acute medical and surgical professionals within the NHS and the UK health regulatory bodies, to provide rapid expert guidance that has saved lives and helped keep the national vaccination programme on track

Post-thrombotic Syndrome: Preventative and Risk Reduction Strategies Following Deep Vein Thrombosis

Venous disease is common in the general population, with chronic venous disorders affecting 50–85% of the western population and consuming 2–3% of healthcare funding. It, therefore, represents a significant socioeconomic, physical and psychological burden. Acute deep vein thrombosis, although a well-recognised cause of death through pulmonary embolism, can more commonly lead to post-thrombotic syndrome (PTS). This article summarises the pathophysiology and risk factor profile of PTS, and highlights various strategies that may reduce the risk of PTS, and the endovenous management of iliofemoral deep vein thrombosis. The authors summarise the advances in PTS risk reduction strategies and present the latest evidence for discussion

Trauma-induced coagulopathy

Acknowledgements E.E.M. and A.S. appreciate the generous support from the National Institutes of Health for their inflammation and coagulation research over the past 35 years (NIGMS: 1-6 P50 GM49222, 1-6 T32 GM08315, 1-2 U54 GM 62119, RM1 GM 131968 and NHLBI: UM1 HL120877). H.B.M. acknowledges the generous support from the National Institutes of Health (NHBLI: K99HL1518870) L.Z.K. acknowledges the generous support from the National Institutes of Health for her platelet biology research (NIGMS: K23GM130892-01). M.D.N. acknowledges the generous support by the National Institutes of Health (NIGMS: R35 GM119526 and NHLBI R01 HL141080).Peer reviewedPostprin

Haematological and fibrinolytic status of Nigerian women with post-partum haemorrhage.

BACKGROUND: Early treatment with tranexamic acid reduces deaths due to bleeding after post-partum haemorrhage. We report the prevalence of haematological, coagulation and fibrinolytic abnormalities in Nigerian women with postpartum haemorrhage. METHODS: We performed a secondary analysis of the WOMAN trial to assess laboratory data and rotational thromboelastometry (ROTEM) parameters in 167 women with postpartum haemorrhage treated at University College Hospital, Ibadan, Nigeria. We defined hyper-fibrinolysis as EXTEM maximum lysis (ML) > 15% on ROTEM. We defined coagulopathy as EXTEM clot amplitude at 5 min (A5)  1.5. RESULTS: Among the study cohort, 53 (40%) women had severe anaemia (haemoglobin< 70 g/L) and 17 (13%) women had severe thrombocytopenia (platelet count < 50 × 109/L). Thirty-five women (23%) had ROTEM evidence of hyper-fibrinolysis. Based on prothrombin ratio criteria, 16 (12%) had coagulopathy. Based on EXTEM A5 criteria, 49 (34%) had coagulopathy. CONCLUSION: Our findings suggest that, based on a convenience sample of women from a large teaching hospital in Nigeria, hyper-fibrinolysis may commonly occur in postpartum haemorrhage. Further mechanistic studies are needed to examine hyper-fibrinolysis associated with postpartum haemorrhage. Findings from such studies may optimize treatment approaches for postpartum haemorrhage. TRIAL REGISTRATION: The Woman trial was registered: NCT00872469; ISRCTN76912190 (Registration date: 22/03/2012)