Genomes and Genes
Affiliation: University College London
- Successful management of pregnancy in women with a history of thrombotic thrombocytopaenic purpuraMarie Scully
Haemostasis Research Unit, Department of Haematology, University College London, UK
Blood Coagul Fibrinolysis 17:459-63. 2006....
- Cryosupernatant and solvent detergent fresh-frozen plasma (Octaplas) usage at a single centre in acute thrombotic thrombocytopenic purpuraM Scully
Haemostasis Research Unit, Department of Haematology, University College London, 51 Chenies Mews, London, UK
Vox Sang 93:154-8. 2007..Thrombotic thrombocytopenic purpura (TTP) is an acute, life-threatening disorder and plasma exchange (PEX) remains the mainstay of treatment...
- Rituximab in the treatment of TTPMarie Scully
University College London Hospitals, London, UK
Hematology 17:S22-4. 2012..The use of ADAMTS 13 activity for monitoring can identify patients at risk of a TTP relapse and preemptive therapy with an anti-CD 20 can be considered...
- Inhibitory anti-ADAMTS 13 antibodies: measurement and clinical applicationMarie Scully
University College Hospital, London, United Kingdom
Blood Rev 24:11-6. 2010..Further investigation into the antibodies produced in TTP and other aspects of immune dysfunction, such as T cells will further our understanding of this devastating disorder...
- Trends in the diagnosis and management of TTP: European perspectiveMarie Scully
Department of Haematology, UCLH, 60 Whitfield Street, London WC1, UK Electronic address
Transfus Apher Sci 51:11-4. 2014..Reviewed is a brief history of TTP and current plasmas available, their use and safety profiles, concentrating on the current UK recommendations. ..
- Human leukocyte antigen association in idiopathic thrombotic thrombocytopenic purpura: evidence for an immunogenetic linkM Scully
Department of Haematology, University College of London Hospitals, and Haemostasis Research Unit, University College London, London, UK
J Thromb Haemost 8:257-62. 2010..The majority of acute, idiopathic, adult TTP cases are associated with anti-ADAMTS 13 IgG antibodies. However, the factor(s) precipitating an acute TTP episode are not always obvious; indeed, a multifactorial etiology is likely...
- A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpuraMarie Scully
Department of Haematology, University College London Hospital, London, United Kingdom
Blood 118:1746-53. 2011..Rituximab should be considered in conjunction with standard therapy on acute presentation of TTP. This study was registered at www.clinicaltrials.gov as NCT009-3713...
- Remission in acute refractory and relapsing thrombotic thrombocytopenic purpura following rituximab is associated with a reduction in IgG antibodies to ADAMTS-13Marie Scully
Department of Haematology, University College London, London, UK
Br J Haematol 136:451-61. 2007..In patients with acute refractory/relapsing idiopathic TTP, rituximab appears to be a safe, effective, targeted therapy with a significant reduction in the requirement for PEX...
- Regional UK TTP registry: correlation with laboratory ADAMTS 13 analysis and clinical featuresMarie Scully
Department of Haematology, Haemostasis Research Unit, University College London, London, UK
Br J Haematol 142:819-26. 2008..Renal impairment and delayed normalisation of platelet count were the main differences between idiopathic and secondary TTP...
- The clinical utility of ADAMTS13 activity, antigen and autoantibody assays in thrombotic thrombocytopenic purpuraRichard Starke
Haemostasis Research Unit, Department of Haematology, University College London, London, UK
Br J Haematol 136:649-55. 2007....
- Acquired, noncongenital thrombotic thrombocytopenic purpura in children and adolescents: clinical management and the use of ADAMTS 13 assaysVickie McDonald
Haemostasis Research Unit, Department of Haematology, University College London, UK
Blood Coagul Fibrinolysis 21:245-50. 2010..Treatment of acquired disease requires PEX and usually immunosuppressive treatment. Rituximab appears to be an effective adjunctive treatment modality...
- Human immunodeficiency virus associated thrombotic thrombocytopenic purpura--favourable outcome with plasma exchange and prompt initiation of highly active antiretroviral therapyDaniel Hart
Department of Haematology, St Bartholomew s and the London Hospital, London, UK
Br J Haematol 153:515-9. 2011..More PEX is required in cases with higher viral loads. Continued HAART maintains remission. In a small proportion of cases, further immunomodulatory therapy may be required...
- Prevalence of the ADAMTS-13 missense mutation R1060W in late onset adult thrombotic thrombocytopenic purpuraR S Camilleri
Haemostasis Research Unit, Department of Haematology, University College London School of Medicine, London, UK
J Thromb Haemost 6:331-8. 2008..ADAMTS-13 mutations and polymorphisms have been reported in childhood congenital TTP, but their significance in adult onset TTP remains unclear...
- Discrepancies between ADAMTS13 activity assays in patients with thrombotic microangiopathiesIan Mackie
Haemostasis Research Unit, Haematology Department, University College London and University College London Hospitals, 1st Floor, 51 Chenies Mews, London, WC1E 6HX, UK
Thromb Haemost 109:488-96. 2013..If the activity does not match the clinical picture, an alternative method should be performed. Where therapeutic monitoring is required, the same activity assay should be used throughout...
- Relationship between ADAMTS13 activity, von Willebrand factor antigen levels and platelet function in the early and late phases after TIA or ischaemic strokeDominick J H McCabe
Department of Neurology, The Adelaide and Meath Hospital incorporating the National Children s Hospital, Trinity College, Dublin, Ireland Stroke Service, The Adelaide and Meath Hospital, Incorporating the National Children s Hospital, Trinity College, Dublin, Ireland Stroke Research Group, UCL Institute of Neurology, The National Hospital for Neurology and Neurosurgery, UK Department of Clinical Neurosciences, Royal Free Campus, UCL Institute of Neurology, The National Hospital for Neurology and Neurosurgery, UK Haemostasis Research Unit, University College, London, UK Electronic address
J Neurol Sci 348:35-40. 2015..ADAMTS13 activity and its relationship with VWF antigen (VWF:Ag) levels and platelet function in 'non-TTP related' TIA or ischaemic stroke has not been comprehensively studied...
- The use of intermediate purity factor VIII concentrate BPL 8Y as prophylaxis and treatment in congenital thrombotic thrombocytopenic purpuraMarie Scully
Haemostasis Research Unit, UCL, London, UK
Br J Haematol 135:101-4. 2006..Effective prophylaxis and treatment is possible in congenital TTP using BPL 8Y...
- Cardiac involvement in acute thrombotic thrombocytopenic purpura: association with troponin T and IgG antibodies to ADAMTS 13C Hughes
Department of Haematology, University College London Hospitals, London, UK
J Thromb Haemost 7:529-36. 2009..Evidence for cardiac involvement in thrombotic thrombocytopenic purpura (TTP) is uncommonly described...
- Thrombotic thrombocytopenic purpura and pregnancy: presentation, management, and subsequent pregnancy outcomesMarie Scully
University College London Hospitals, London, United Kingdom
Blood 124:211-9. 2014..cTTP presents more frequently than acquired TTP during pregnancy and must be differentiated by ADAMTS13 analysis. Careful diagnosis, monitoring, and treatment in congenital and acquired TTP have assisted in excellent pregnancy outcomes. ..
- Complement and cytokine response in acute Thrombotic Thrombocytopenic PurpuraJohn Paul Westwood
Haemostasis Research Unit, University College London, London, UK
Br J Haematol 164:858-66. 2014..These results suggest complement anaphylatoxin levels are higher in acute TTP cases than in remission, and the complement response seen acutely may relate to anti-ADAMTS13 IgG antibody and IL-10 levels...
- Thrombotic thrombocytopaenic purpura in HIV-infected patientsR F Miller
Centre for Sexual Health and HIV Research, Department of Primary Care and Population Sciences, Royal Free and University College Medical School, University College London, Mortimer Market Centre, Camden PCT, London WC1E 6AU, UK
Int J STD AIDS 16:538-42. 2005..Given the clear benefit from HAART in addition to plasma exchange, these data suggest that all patients presenting with TTP should undergo HIV testing...
- The impact of dose escalation and resistance modulation in older patients with acute myeloid leukaemia and high risk myelodysplastic syndrome: the results of the LRF AML14 trialAlan K Burnett
Department of Haematology, School of Medicine, Cardiff University Heath Park, Cardiff, UK
Br J Haematol 145:318-32. 2009..In conclusion, these four interventions have not improved outcomes in older patients. New agents need to be explored and novel trial designs are required to maximise prospects of achieving timely progress...