Table 1. Classification of mast cell mediated diseases*
1. Primary (Intrinsic)
a. Mastocytosis
b. Monoclonal mast cell activation syndrome (MMAS)
c. Autosomal dominant vibratory urticaria (VU)
d. Hereditary alpha tryptasemia (HaT)
2. Secondary (Extrinsic)
a. Allergic disorders (FcεRI mediated)
b. Mast cell activation through the high affinity IgG receptor (CD64)
c. MRGPRX2 mediated reactions
d. Diseases associated with complement activation (C3a, C5a)
3. Idiopathic#
a. Anaphylaxis
b. Angioedema
c. Urticaria
d. Idiopathic mast cell activation syndrome (Idiopathic MCAS)
*See text for explanation. Focus is on human mast cell-associated diseases, examples only
# When no inciting stimulus is identified
Table 2 . KIT-independent constitutive and inducible mouse models of MC deficiency.