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, ANNEXES Annexe 1. Critères diagnostiques de Mastocytose systémique d' après Barete, p.89
, Critère majeur
, Infiltrats denses et multifocaux de mastocytes au niveau de la moelle osseuse ou d'autres organes extracutanés
, Critères mineurs
, Présence de mastocytes atypiques de forme allongés dans la moelle (>25%)
, Coexpression du CD2 et/ou du CD25 par les mastocytes médullaires, sanguins ou d'autres organes extracutanés
, Mutation du C-KIT sur le codon 816 dans la moelle osseuse, le sang ou d'autres organes extracutanés
, Tryptase sérique > 20µg/L
, Le diagnostic est établi en présence d'un critère majeur et un critère mineur ou de trois critères mineurs
, Symptômes associés à la mastocytose selon le score AFIRMM
, Chaque handicap
, Le score AFIRMM a ensuite été calculé comme suit: où n est le numéro du symptôme, Grade est la gravité auto-évaluée du symptôme (0-4), et Weight est la pondération
, Le score AFIRMM peut aller d'un minimum de 0 (aucun handicap) jusqu'à un maximum de 760
, Annexe 3. Score SCORMA d'après Heide et al.(60)