Anaphylaxis and Mast Cell Disease: What Is the Risk?

Abstract

Mastocytosis is a proliferative disorder of the hematopoietic mast cell progenitor that results from expansion of a clone carrying the D816V c-kit mutation. Based on the dramatic increase in incidence of anaphylaxis in patients with mastocytosis, recent studies analyzed the presence of clonal mast cell markers, including D816V c-kit mutation, in patients with recurrent IgE- and non-IgE-mediated anaphylaxis. These studies demonstrated the presence of an aberrant mast cell clone in a significant proportion of patients with unexplained anaphylaxis, or anaphylaxis due to hymenoptera venom. Clonal mast cell disease should be suspected in particular in patients presenting with profound cardiovascular manifestations such as hypotension and syncope in the absence of urticaria.

Primary role of multiparametric flow cytometry in the diagnostic work‐up of indolent clonal mast cell disorders

“…The true incidence of CMD in patients with inappropriate mediator release symptoms, such severe anaphylactic reactions, or unexplained osteoporosis without skin involvement is probably underestimated (7,(10)(11)(12)27). The diagnosis of these patients requires a multidisciplinary approach.…”

“…The term Monoclonal MC Activation Syndrome (MMAS) has been proposed to identify the subjects with unexplained or recurrent anaphylaxis without skin lesions and in the absence of the major criterion, but with documented clonality markers (7,(10)(11)(12). Altogether, SM and MMAS may be grouped as systemic clonal MC disorders (CMD).…”

Risk factors for anaphylaxis in patients with mastocytosis

“…4 It is believed that mast cells in mastocytosis patients may have an intrinsic defect lowering the threshold for activation and/or increasing its sensitivity to allergens. 3 Anaphylactic reactions occur in 30% of all patients with mastocytosis and in 50% of patients with systemic mastocytosis (SM). 3,5,6 Although the incidence of anaphylactic reactions (both immunoglobulin E [IgE]-dependent and IgE-independent) in patients with mastocytosis is significantly higher than in the general population, the frequency of atopy is similar in both populations.…”

“…3 Anaphylactic reactions occur in 30% of all patients with mastocytosis and in 50% of patients with systemic mastocytosis (SM). 3,5,6 Although the incidence of anaphylactic reactions (both immunoglobulin E [IgE]-dependent and IgE-independent) in patients with mastocytosis is significantly higher than in the general population, the frequency of atopy is similar in both populations. 7 Insect stings are considered a major cause of mast cell activation in patients with mastocytosis.…”

Drug hypersensitivity reactions and allergy in patients with mastocytosis

“…Szacuje się, że 30-50% dorosłych chorych doświadcza reakcji anafilaktycznych [10,12,14], natomiast u dzieci chorujących na mastocytozę częstość reakcji anafilaktycznych oceniana jest na 5,5-9% [3,5,10]. Objawy zależne od mediatorów oraz ciężkie reakcje anafilaktyczne u chorych na mastocytozę mogą być wyzwalane przez różnorodne czynniki, takie jak: jady owadów błonkoskrzydłych, leki, pokarmy, alkohol, stres emocjonalny, wysiłek fizyczny, zabiegi inwazyjne, zakażenia, czynniki fizyczne oraz niezidentyfikowane czynniki [9][10][11][12][14][15][16][17][18][19][20][21][22][23][24][25]. Według danych z piśmiennictwa nie tylko częstość występo-wania, lecz także czynniki wyzwalające oraz indywidualne ryzyko wystąpienia objawów zależnych od mediatorów oraz reakcji anafilaktycznych różnią się u dzieci i u dorosłych chorujących na mastocytozę [5,10,12,16,17].…”