Publications
347 results found
Suiter T, Mannucci PM, Kempton CL, et al., 2013, Detection of non inhibitory binding antibodies to von Willebrand factor affecting the clearance of VWF: AG in von willebrand disease, JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Vol: 11, Pages: 97-98, ISSN: 1538-7933
Shapiro S, Laffan M, 2013, New oral anticoagulants: their role and future., Clin Med (Lond), Vol: 13 Suppl 6, Pages: s53-s57
After 60 years in which warfarin has been the only practical oral anticoagulant, a number of new oral anticoagulants are entering practice. These drugs differ in a several important respects from warfarin; most notably they have a reliable dose-response effect which means they can be given without the need for monitoring. Their simpler metabolism and mode of action also results in fewer interactions with other drugs and with diet. However, some of their other properties such as renal clearance (to varying degrees), short half-life and lack of an available antidote may slow their rate of introduction. Large trials have established their non-inferiority to warfarin in a number of indications and in some cases their superiority. To date they have been licensed for prophylaxis following high risk orthopaedic procedures, non-valvular atrial fibrillation and treatment of venous thromboembolism, but is not clear that they will supplant warfarin in all areas.
Randi AM, Laffan MA, Starke RD, 2013, Von Willebrand factor, angiodysplasia and angiogenesis, Mediterranean Journal of Hematology and Infectious Diseases, Vol: 5, ISSN: 2035-3006
The large multimeric glycoprotein Von Willebrand factor (VWF) is best known for its role in haemostasis; however in recent years other functions of VWF have been identified, indicating that this protein is involved in multiple vascular processes. We recently described a new role for VWF in controlling angiogenesis, which may have significant clinical implications for patients with Von Willebrand disease (VWD), a genetic or acquired condition caused by the deficiency or dysfunction of VWF. VWD can be associated with angiodysplasia, a condition of degenerative blood vessels often present in the gastrointestinal tract, linked to dysregulated angiogenesis. Angiodysplasia can cause severe intractable bleeding, often refractory to conventional VWD treatments. In this review we summarise the evidence showing that VWF controls angiogenesis, and review the angiogenic pathways which have been implicated in this process. We discuss the possible mechanisms though which VWF regulates angiopoietin-2 (Ang-2) and integrin αvβ3, leading to signalling through vascular endothelial growth factor receptor-2 (VEGFR2), one of the most potent activators of angiogenesis. We also review the evidence that links VWF with angiodysplasia, and how the newly identified function of VWF in controlling angiogenesis may pave the way for the development of novel therapies for the treatment of angiodysplasia in congenital VWD and in acquired conditions such as Heyde syndrome.
Chambost H, Santagostino E, Laffan M, et al., 2013, Real-world outcomes with recombinant factor VIIa treatment of acute bleeds in haemophilia patients with inhibitors: results from the international ONE registry, HAEMOPHILIA, Vol: 19, Pages: 571-577, ISSN: 1351-8216
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- Citations: 22
Patel A, Laffan MA, 2013, Transfusion for Acute Upper Gastrointestinal Bleeding, New England Journal of Medicine
Paramasivan P, Venkatesh SP, Vyas V, 2013, Transfusion for acute upper gastrointestinal bleeding., N Engl J Med, Vol: 368, Pages: 1361-1362
Manickam P, Kanaan Z, Cappell MS, 2013, Transfusion for acute upper gastrointestinal bleeding., N Engl J Med, Vol: 368
Villanueva C, Colomo A, Bosch A, 2013, Transfusion for Acute Upper Gastrointestinal Bleeding Reply, NEW ENGLAND JOURNAL OF MEDICINE, Vol: 368, Pages: 1362-1363, ISSN: 0028-4793
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- Citations: 27
Starke RD, Paschalaki KE, Dyer CE, et al., 2013, Cellular and molecular basis of von Willebrand disease: studies on blood outgrowth endothelial cells, Blood, Vol: 121, Pages: 2773-2784
Von Willebrand disease (VWD) is a heterogeneous bleeding disorder caused by decrease or dysfunction of von Willebrand factor (VWF). A wide range of mutations in the VWF gene have been characterized; however, their cellular consequences are still poorly understood. Here we have used a recently developed approach to study the molecular and cellular basis of VWD. We isolated blood outgrowth endothelial cells (BOECs) from peripheral blood of 4 type 1 VWD and 4 type 2 VWD patients and 9 healthy controls. We confirmed the endothelial lineage of BOECs, then measured VWF messenger RNA (mRNA) and protein levels (before and after stimulation) and VWF multimers. Decreased mRNA levels were predictive of plasma VWF levels in type 1 VWD, confirming a defect in VWF synthesis. However, BOECs from this group of patients also showed defects in processing, storage, and/or secretion of VWF. Levels of VWF mRNA and protein were normal in BOECs from 3 type 2 VWD patients, supporting the dysfunctional VWF model. However, 1 type 2M patient showed decreased VWF synthesis and storage, indicating a complex cellular defect. These results demonstrate for the first time that isolation of endothelial cells from VWD patients provides novel insight into cellular mechanisms of the disease
Starke RD, Paschalaki KE, Dyer CEF, et al., 2013, Cellular and molecular basis of von Willebrand disease: studies on blood outgrowth endothelial cells, BLOOD, Vol: 121, Pages: 2773-2784, ISSN: 0006-4971
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- Citations: 69
Mackie I, Cooper P, Lawrie A, et al., 2013, Guidelines on the laboratory aspects of assays used in haemostasis and thrombosis, INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Vol: 35, Pages: 1-13, ISSN: 1751-5521
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- Citations: 123
Shapiro SE, Phillips E, Manning RA, et al., 2013, Clinical phenotype, laboratory features and genotype of 35 patients with heritable dysfibrinogenaemia, BRITISH JOURNAL OF HAEMATOLOGY, Vol: 160, Pages: 220-227, ISSN: 0007-1048
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- Citations: 62
Makris M, Van Veen JJ, Tait CR, et al., 2013, Guideline on the management of bleeding in patients on antithrombotic agents, BRITISH JOURNAL OF HAEMATOLOGY, Vol: 160, Pages: 35-46, ISSN: 0007-1048
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- Citations: 151
Starke RD, Paschalaki KE, Dyer C, et al., 2012, Cellular and molecular basis of Von Willebrand disease: Studies on blood outgrowth endothelial cells, Atherosclerosis, Vol: 225, Pages: e5-e6, ISSN: 0021-9150
Canis K, McKinnon TAJ, Nowak A, et al., 2012, Mapping the N-glycome of human von Willebrand factor, BIOCHEMICAL JOURNAL, Vol: 447, Pages: 217-228, ISSN: 0264-6021
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- Citations: 76
Tait C, Baglin T, Watson H, et al., 2012, Guidelines on the investigation and management of venous thrombosis at unusual sites, BRITISH JOURNAL OF HAEMATOLOGY, Vol: 159, Pages: 28-38, ISSN: 0007-1048
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- Citations: 104
Al-Jafar H, Laffan M, Al-Sabah S, et al., 2012, Severe recurrent achalasia cardia responding to treatment of severe autoimmune acquired haemophilia, Case Reports in Gastroenterology, Vol: 6, Pages: 618-623, ISSN: 1662-0631
Acquired haemophilia A and severe acquired achalasia are both very rare conditions with unknown aetiology. Haemophilia A is a haemorrhagic disease induced by deficiency or malfunction of coagulation factor VIII. Congenital haemophilia is an inherited disease transmitted by the mother through X-linked inheritance and primarily affects males. However, acquired haemophilia A is a serious, sudden-onset, autoimmune disease that affects either sex. In addition, achalasia is a disease of the oesophagus caused by abnormal function of the nerves and muscles. It causes swallowing difficulties due to the inability of the lower oesophageal sphincter to relax during swallowing, leading to dysphagia, regurgitation and chest pain. In this report, we describe the case of a patient with severe, newly diagnosed, acquired haemophilia A with long-standing, recurrent achalasia; the achalasia had recurred 3 times despite complete and proper surgical fixation. Acquired haemophilia A is treated with immunosuppressive therapy. High-dose steroid therapy was administered for 7 months, during which the patient responded well; moreover, the achalasia did not recur for more than 2 years. The response of the achalasia to immunosuppressive therapy suggests that achalasia may be an autoimmune disorder and that there may be an association between both diseases. The findings of the present case suggest that achalasia may favourably respond to steroid therapy as a first-line treatment prior to surgery.
Berntorp E, Peake I, Budde U, et al., 2012, von Willebrand's disease: a report from a meeting in the Aland islands, HAEMOPHILIA, Vol: 18, ISSN: 1351-8216
Neelakantan P, Marin D, Laffan M, et al., 2012, Platelet dysfunction associated with ponatinib, a new pan BCR-ABL inhibitor with efficacy for chronic myeloid leukemia resistant to multiple tyrosine kinase inhibitor therapy, HAEMATOLOGICA-THE HEMATOLOGY JOURNAL, Vol: 97, Pages: 1444-1444, ISSN: 0390-6078
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- Citations: 30
Everett R, Ahmed S, Laffan M, 2012, Abdominal pain in a patient with haemophilia and metallic valve replacement, HAEMOPHILIA, Vol: 18, Pages: E370-E371, ISSN: 1351-8216
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- Citations: 2
Nowak AA, Canis K, Riddell A, et al., 2012, <i>O</i>-linked glycosylation of von Willebrand factor modulates the interaction with platelet receptor glycoprotein Ib under static and shear stress conditions, BLOOD, Vol: 120, Pages: 214-222, ISSN: 0006-4971
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- Citations: 50
Shapiro S, Laffan M, 2012, Making contact with microparticles, JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Vol: 10, Pages: 1352-1354, ISSN: 1538-7933
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- Citations: 3
McKinnon TAJ, Nowak AA, Cutler J, et al., 2012, Characterisation of von Willebrand factor A1 domain mutants I1416N and I1416T: correlation of clinical phenotype with flow-based platelet adhesion, JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Vol: 10, Pages: 1409-1416, ISSN: 1538-7933
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- Citations: 5
Bentley P, Rosso M, Sadnicka A, et al., 2012, Intravenous immunoglobulin increases plasma viscosity without parallel rise in blood pressure, JOURNAL OF CLINICAL PHARMACY AND THERAPEUTICS, Vol: 37, Pages: 286-290, ISSN: 0269-4727
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- Citations: 18
Ranger A, Manning RA, Lyall H, et al., 2012, Pregnancy in type 2B VWD: a case series, HAEMOPHILIA, Vol: 18, Pages: 406-412, ISSN: 1351-8216
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- Citations: 15
Mahlangu JN, Coetzee MJ, Laffan M, et al., 2012, Phase I, randomized, double-blind, placebo-controlled, single-dose escalation study of the recombinant factor VIIa variant BAY 86-6150 in hemophilia, JOURNAL OF THROMBOSIS AND HAEMOSTASIS, Vol: 10, Pages: 773-780, ISSN: 1538-7933
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- Citations: 34
Beltran-Miranda CP, Luna-Zaizar H, Lopez-Jimenez JJ, et al., 2012, Phenotypic variability in a family with haemophilia B and prothrombin G20210A, HAEMOPHILIA, Vol: 18, Pages: E22-E23, ISSN: 1351-8216
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- Citations: 1
Livesey JA, Manning RA, Meek JH, et al., 2011, Low serum iron levels are associated with elevated plasma levels of coagulation factor VIII and pulmonary emboli/deep venous thromboses in replicate cohorts of patients with hereditary haemorrhagic telangiectasia., Thorax, Vol: 67, Pages: 328-333, ISSN: 0040-6376
BackgroundElevated plasma levels of coagulation factor VIII are a strong risk factor for pulmonary emboli and deep venous thromboses.ObjectivesTo identify reversible biomarkers associated with high factor VIII and assess potential significance in a specific at-risk population.Patients/Methods609 patients with hereditary haemorrhagic telangiectasia were recruited prospectively in two separate series at a single centre. Associations between log-transformed factor VIII measured 6 months from any known thrombosis/illness, and patient-specific variables including markers of inflammation and iron deficiency, were assessed in stepwise multiple regression analyses. Age-specific incidence rates of radiologically proven pulmonary emboli/deep venous thromboses were calculated, and logistic regression analyses performed.ResultsIn each series, there was an inverse association between factor VIII and serum iron that persisted after adjustment for age, inflammation and/or von Willebrand factor. Iron response elements within untranslated regions of factor VIII transcripts provide potential mechanisms for the association. Low serum iron levels were also associated with venous thromboemboli (VTE): the age-adjusted OR of 0.91 (95% CI 0.86 to 0.97) per 1 μmol/litre increase in serum iron implied a 2.5-fold increase in VTE risk for a serum iron of 6 μmol/litre compared with the mid-normal range (17 μmol/litre). The association appeared to depend on factor VIII, as once adjusted for factor VIII, the association between VTE and iron was no longer evident.ConclusionsIn this population, low serum iron levels attributed to inadequate replacement of haemorrhagic iron losses are associated with elevated plasma levels of coagulation factor VIII and venous thromboembolic risk. Potential implications for other clinical populations are discussed.
Franchini M, Targher G, Lippi G, 2011, Safety of recombinant activated factor VII in randomized clinical trials., N Engl J Med, Vol: 364
Arellano-Rodrigo E, 2011, Safety of recombinant activated factor VII in randomized clinical trials., N Engl J Med, Vol: 364, Pages: 574-575
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