Réseau Amylose Mondor et Centre de Référence National des Amyloses Cardiaques

Etude des immunoglobulines

L’amylose peut-être liée à des dépôts dans les organes de substances dérivées des immunoglobulines. Les immunoglobulines sont des protéines de défense immunitaire produites par des cellules de la moelle osseuse : les plasmocytes. Chaque individu produit des milliards d’immunoglobulines différentes pour lutter contre tous les types d’infection possible. Il arrive qu’une population de plasmocytes se multiplie en excès et produise une immunoglobuline particulière, dont les propriétés physico-chimiques permettent l’accumulation dans les tissus de soutien d’organes comme le cœur, les reins ou les nerfs. C’est souvent une partie de l’immunoglobuline : la « chaîne légère » qui s’accumule et créé les lésions dans l’organisme.

Au laboratoire d’immunologie, nous pouvons détecter dans le sang une immunoglobuline produite en excès et la quantifier. Nous pouvons également doser les chaînes légères d’immunoglobulines dans le sang et les détecter dans les urines.

Pour cela nous utilisons des techniques de migration des protéines dans un champ électrique (électrophorèse) et des techniques dites « immunologiques » : immunofixation, immunonéphélémétrie. Ces différentes techniques sont complémentaires et permettent le diagnostic et le suivi sous traitement de l’amylose. En effet, le succès du traitement s’accompagne d’une diminution de l’immunoglobuline et/ou de la chaîne légère libre associée à la maladie.

 


 

Electrophorèse des protéines et immunofixation


(A) Les protéines du sérum ont été migrées selon leurs propriétés physicochimiques dans un champ électrique (deux techniques sont actuellement disponibles : électrophorèse capillaire qui permet d’obtenir d’emblée un tracé de migration et électrophorèse en gel d’agarose avec coloration des protéines, tel que visualisé dans l’insert). Les 6 fractions de droite à gauche sont l’albumine, les a1-globulines, les a2-globulines, les b1-globulines, les b2-globulines et les g-globulines. Un pic évocateur d’immunoglobuline monoclonale est visible sur les deux tracés de droite.
 
(B) La technique d’immunofixation sur gel permet d’identifier l’immunoglobuline correspondant au pic. Sur la piste de gauche, une électrophorèse des protéines totales permet de visualiser les fractions. Sur les pistes suivantes les protéines sont immunofixées après migration par un anticorps spécifique de chaque chaîne lourde (anti-g, anti-a, anti-µ) et légère (anti-k, anti-l) d’immunoglobuline. Du fait de leur rareté, les IgD et IgE ne sont pas recherchées en première intention. A gauche, un aspect normal, au centre, une IgG k monoclonale est détectée, à droite, une IgA l monoclonale est détectée.

 

 

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