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Coombs-positive Paroxysmal Nocturnal Haemoglobinuria
Sabrina Kalam1, Rupert Beale2, Derralynn Hughes3
1Department of Neurology, Charing Cross Hospital, London, W6 8RF, UK.
Oxford Medical Case Reports
|April 8, 2020
Summary
Autoimmune haemolytic anaemia (AIHA) and paroxysmal nocturnal haemoglobinuria (PNH) can co-occur. A comprehensive work-up is crucial for diagnosing concurrent AIHA and PNH in patients with haemolytic anaemia.
Area of Science:
- Hematology
- Immunology
- Internal Medicine
Background:
- Autoimmune haemolytic anaemia (AIHA) and paroxysmal nocturnal haemoglobinuria (PNH) are distinct hemolytic anemias with different pathogenic mechanisms and treatment approaches.
- The direct antiglobulin test (DAT), or Coombs test, is a key diagnostic tool, positive in AIHA but negative in PNH.
Observation:
- A case study involving a female patient presenting with unexplained hemolytic anemia.
Findings:
- The patient exhibited concurrent evidence of both AIHA and PNH.
- This dual diagnosis challenges typical diagnostic pathways based on DAT results.
Implications:
- Highlights the necessity of a thorough and comprehensive hemolysis work-up for all patients with hemolytic anemia.
- Suggests that standard diagnostic algorithms may need re-evaluation to account for rare co-occurring conditions.
- Emphasizes the importance of considering multiple etiologies in complex hematological presentations.
Keywords:
Autoimmune haemolytic anaemiaDirect antiglobulin testEculizumabGlycosylphosphatidylinositolPIGAParoxysmal Nocturnal HaemoglobinuriaMore Related Videos
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