BioTribune Magazine

, Volume 27, Issue 1, pp 28–31 | Cite as

Surveillance et diagnostic de l’hémoglobinurie paroxystique nocturne en cytométrie de flux

Dossier

Résumé

L’hémoglobinurie paroxystique nocturne est une maladie liée à une mutation somatique entraînant l’expansion de clones cellulaires déficients pour des molécules d’ancrage de protéines de surface cellulaire. Plusieurs formes d’expression de ces mutations peuvent être rencontrées avec des implications diagnostiques et thérapeutiques différentes. La technologie recommandée pour poser le diagnostic et surveiller les patients, est la cytométrie de flux. Cependent l’approche technologique est différente, s’il s’agit de diagnostiquer une hémoglobinurie paroxystique nocturne (HPN) classique ou maladie de Machiavela-Micheli, ou identifier une petite souspopulation associée à une insuffisance médullaire, la pathologie dans ce cas intéressant des cellules non HPN.

Mots clés

Hémoglobinurie paroxystique nocturne Cytométrie de flux CD55 CD59 Syndromes myélodysplasiques 

Traduction anglaise du titre à fournir

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a disease linked to a somatic mutation which is accompanied by expansion of cell clones deficient in molecules needed to make cell membrane anchors for proteins. These mutations can be found in diverse forms, resulting in differing diagnostic and therapeutic implications. Flow cytometry is the recommended technology for confirming diagnosis and monitoring patients. However, the technological approach differs according to whether the aim is to diagnose classic PNH (Marchiafava-Micheli syndrome) or identify one of the sub-populations that can occur in cases of bone marrow failure, where the disease affects PNH cells.

Keywords

Paroxysmal nocturnal hemoglobinuria Flow cytometry CD55 CD59 Myelodysplasic syndromes 

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Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  1. 1.Institut Biotechnologique de TroyesTroyesFrance
  2. 2.HématologieCentre Hospitalier de TroyesTroyesFrance

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