1. Diagnostic uncertainty presented barriers to the timely management of acute thrombotic thrombocytopenic purpura in the United Kingdom between 2014 and 2019
- Author
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Tom P. Bull, Rory McCulloch, Phillip L.R. Nicolson, Andrew J. Doyle, Rebecca J. Shaw, Alexander Langridge, Zara Sayar, David L. Tucker, Michala Pettit, Rita Perry, William Thomas, Catherine Page, Ioana Whalley, Tina Dutt, Louise Garth, Will Lester, Richard J. Buka, Mary Subhan, Victoria Ware, Rachel Rayment, Daniel Castle, Astrid Etherington, Luke Carter‐Brzezinski, Jayne Peters, Claire Corrigan, Narind Sharma, Gary Benson, Sarah Challenor, Thomas S. Skinner, Rui Zhao, Lyndsay A.G. McLeod‐Kennedy, Kenneth Douglas, Amy Knott, Sophie Smith, Julia Wolf, Sophie A. Todd, Vickie McDonald, Alexandros Rampotas, Christopher Dean, Gavinda Sangha, Sue Pavord, Nicholas Denny, Sarah Jaafar, David P.T. McLaughlin, Jennifer E. Ross, Mamatha Karanth, Sarah L. Beverstock, Lynn Mansonso, Samuel H. Burrows, Sudhir Tauro, Amir Shenouda, Benjamin M. Bailiff, Daniel Kajita, Joannes Hermans, Harshita Goradia, Emily M. Finan, Sarah Alford, Keir Pickard, Brigit Greystoke, Thomas Fail, Asmaa Abdussalam, Lara N Roberts, James B. Clark, Natalie Heeney, Jennifer Young, Jamie Maddox, Swathy Srinath, Jahanzeb Khawaja, Jayne Parkes, Samah Babiker, Beverley J. Hunt, Sarah L. Wheeldon, Paul Kerr, Molham Tahhan, Mark Vickers, Alexandra C. Pike, Quentin Hill, Nadreen Mustafa, Azza Almaremi, Emily Hughes, Sean J.F. McGoldrick, Eleana Loizou, Izabela James, Sara R. Boyce, Isabel Farmer, Murugaiyan Thanigaikumar, Katherine Wickenden, Richard Gooding, Kathryn Thornton, Clare Kane, Adam Cole, JessicaC Griffin, Suzanne Docherty, Kiri I. Dixon, Josephine Crowe, Mathew Sheridan, Corinne De Lord, Amit Sud, Anna Austin, Nichola Coooper, Chris Bailey, Luke Attwell, Rachel Hall, Benjamin Gray, Salena R. Chauhan, Anand Lokare, Amy Gudger, Claire Horgan, Indrani Venkatadasari, Israa Kaddam, Claire L. Mapplebeck, Joost Van Veen, Maya Raj, Kanchana De Abrew, Edward Belsham, Cecilia Gyansah, Shalal Sadullah, Beena Salhan, Richard Murrin, Rhys L. Williams, Andrew Stewart, Naomi Cornish, Sophie Otton, Zeeshan Khan, Sam Ackroyd, Lucia Y. Chen, Nicholas P. Lafferty, Francesca Leonforte, Nicholas Pemberton, Emanal Rawi, Diana Triantafyllopoulou, Jagdish Adiyodi, Jun Yong, Elizabeth Jones, David Davies, Rachel C. Peck, Robson Philip, Thomas Seddon, Paul Cahalin, Catherine Prodger, David A. Dutton, Alexander J. Sternberg, Rajani Chengal, Paolo Polzella, and Marie Scully
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Adult ,Treatment Outcome ,Plasma Exchange ,Purpura, Thrombotic Thrombocytopenic ,Uncertainty ,Humans ,Hematology ,Retrospective Studies - Abstract
Acute thrombotic thrombocytopenic purpura (TTP) is a life-threatening emergency and plasma exchange (PEX) is the initial treatment shown to reduce acute mortality.To compare current practice in the United Kingdom (UK) against the standards set out in the 2012 British Society of Haematology guideline, and to better understand the issues affecting prompt initiation of PEX.The trainee research network HaemSTAR conducted a retrospective nationwide review of adults presenting to UK hospitals with a first episode of acute TTP.Data on 148 patients treated at 80 UK hospitals between 2014 and 2019 demonstrated that 64.8% of patients received PEX within 24 h. Diagnostic uncertainty was the most commonly cited reason for delayed treatment. Conversely, a shorter time to PEX occurred in patients who had red cell fragments or severe thrombocytopenia identified on their first complete blood count. Availability of on-site PEX was associated with a greater proportion of patients receiving PEX within 8 h compared to patients transferred, but by 24 h there was no difference between the two groups and two-thirds of all patients had received their first PEX. The mortality rate for patients that received PEX was 9.2%, with 27.8% of deaths linked to delayed treatment initiation.This is the first multi-center evaluation of treatment delays in acute TTP and it will inform targeted pathways to improve prompt access to life-saving intervention.
- Published
- 2022
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