Conclusion
Extract from the VADE-MECUM by Professor Gérard Socié
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. PNH is a debilitating disease in young people and can be fatal.
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. Chronic haemolysis mediated by the complement is the cause of morbi-mortality in PNH.
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. Thrombosis is the leading cause of death in PNH.
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. 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.
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. Flow cytometry is the reference method for diagnosis.
- . Regular LDH monitoring is crucial to assess the extent of haemolysis.
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