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Conclusion

Extrait du VADE-MECUM, Professeur Gérard Socié

  • L’HPN est une maladie invalidante du sujet jeune pouvant menacer le pronostic vital.

  • L’hémolyse chronique médiée par le complément est la cause de la morbi-mortalité de l’HPN.

  • La thrombose est la principale cause de décès dans l’HPN. 

  • Les présentations cliniques justifiant une recherche de clone HPN sont : une anémie hémolytique à test de Coombs négatif, une hémoglo­binurie, une thrombose inexpliquée, une aplasie médullaire, un syndrome myélodys­plasique avec anémie réfractaire, une cytopénie inexpliquée.

  • La cytométrie en flux est la méthode de référence pour le diagnostic.

  • . Le contrôle régulier des LDH est déterminant pour évaluer l’intensité de l’hémolyse.

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la comparaison interlaboratoire

L'observatoire national des clones HPN

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