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Coombs-positive Paroxysmal Nocturnal Haemoglobinuria

Kalam, S; Beale, R; Hughes, D; Kulasekararaj, A; Srirangalingam, U; (2020) Coombs-positive Paroxysmal Nocturnal Haemoglobinuria. Oxford Medical Case Reports (3) , Article omz125. 10.1093/omcr/omz125. Green open access

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Abstract

Autoimmune haemolytic anaemia (AIHA) and paroxysmal nocturnal haemoglobinuria (PNH) are two distinct causes of haemolytic anaemia. They have different mechanisms that underpin their pathogenesis and, therefore, require different treatment strategies. The direct antiglobulin test (DAT) or Coombs test is positive in cases of immune-mediated haemolytic anaemia and, thus, is positive in AIHA but negative in PNH. We report a case of a woman presenting with a haemolytic anaemia who was found to have concomitant evidence of AIHA and PNH. The case highlights the importance of carrying out a comprehensive haemolysis work-up in patients who present with haemolytic anaemia.

Type: Article
Title: Coombs-positive Paroxysmal Nocturnal Haemoglobinuria
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1093/omcr/omz125
Publisher version: https://doi.org/10.1093/omcr/omz125
Language: English
Additional information: © The Author(s) 2020. Published by Oxford University Press. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/).
Keywords: Autoimmune haemolytic anaemia, Direct antiglobulin test, Eculizumab, Glycosylphosphatidylinositol, PIGA, Paroxysmal Nocturnal Haemoglobinuria
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute > Research Department of Haematology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Renal Medicine
URI: https://discovery.ucl.ac.uk/id/eprint/10096815
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