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Extract from the VADE-MECUM by Professor Gérard Socié

  • PNH is a debilitating disease in young people and can be fatal.

  • Chronic haemolysis mediated by the complement is the cause of morbi-mortality in PNH.

  • Thrombosis is the leading cause of death in PNH.

  • The clinical presentations justifying tests for the PNH clone are: Coombs-negative haemolytic anaemia, haemoglobinuria, unexplained thrombosis, bone marrow failure, myelodysplastic syndrome with refractory anaemia and unexplained cytopenia.

  • Flow cytometry is the reference method for diagnosis.

  • . Regular LDH monitoring is crucial to assess the extent of haemolysis.


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