Clonal mast cell disorders in patients with severe Hymenoptera venom allergy and normal serum tryptase levels

Abstract

The absence of urticaria or angioedema in severe reactions to Hymenoptera stings with hypotension might represent the most relevant factor in identifying patients with mastocytosis, regardless of their serum tryptase levels.

Risk factors and indicators of severe systemic insect sting reactions

“…The frequent absence of urticaria/angioedema during severe, venom‐induced anaphylaxis has been attributed to either counter‐regulatory release of endogenous epinephrine or preferential activation of cardiac mast cells . The complete lack of cutaneous symptoms, or flushing rather than urticaria/angioedema, is associated with increased tryptase levels and was recently suggested to be an even stronger predictor of mast cell clonality than tryptase elevation itself in venom‐allergic patients with severe, hypotensive sting reactions . The REMA score as proposed by the Red Española de Mastocitosis (Spanish Network on Mastocytosis) for screening patients for the necessity of bone marrow biopsy accordingly includes the absence of urticaria/angioedema during anaphylaxis as a predictor of bone marrow mast cell clonality along with male sex, hypotensive shock, and tryptase elevation >25 ng/mL …”

Key Issues in Hymenoptera Venom Allergy: An Update

“…Clonal mast cell activation syndrome is considered a risk factor both for experiencing SR in VIT and for severe and fatal reactions with a subsequent sting after interrupting treatment [49,68,69]. However, in the absence of mastocytosis, the association between BST and lack of efficacy of VIT is controversial.…”

À qui prescrire un dispositif auto-injectable d’adrénaline ? Position des groupes de travail « Anaphylaxie », « Allergie alimentaire », « Insectes piqueurs » sous l’égide de la Société française d’allergologie

“…Elle est aussi élevée, de 20 à 30 %, parmi les patients allergiques aux venins d'hyménoptères [23]. Dans l'étude de Bonadonna et al, 16 des 22 patients aux antécédents d'anaphylaxie liée aux venins d'hyménoptères avec hypotension et un taux de tryptase sérique de base normal (< 11,4 ng/mL) avaient néanmoins une mastocytose objectivée par une biopsie de moelle osseuse [24].…”