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Case Reports
. 2020 Mar 30;2020(3):omz125.
doi: 10.1093/omcr/omz125. eCollection 2020 Mar.

Coombs-positive Paroxysmal Nocturnal Haemoglobinuria

Sabrina Kalam  1 , Rupert Beale  2 , Derralynn Hughes  3 , Austin Kulasekararaj  4 , Umasuthan Srirangalingam  5
Affiliations
Case Reports

Coombs-positive Paroxysmal Nocturnal Haemoglobinuria

Sabrina Kalam et al. Oxf Med Case Reports. .

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.

Keywords: Autoimmune haemolytic anaemia; Direct antiglobulin test; Eculizumab; Glycosylphosphatidylinositol; PIGA; Paroxysmal Nocturnal Haemoglobinuria.

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Figures

Figure 1
Figure 1
Blood film appearances showing polychromasia (large bluish red cells) and spherocytes (small round red cells with no central pallor).
See this image and copyright information in PMC

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