Systemic mastocytosis | Monoclonal mast cell activation syndrome (MMAS) | Idiopathic mast cell activation syndrome (IMCAS) | Idiopathic anaphylaxis | |
Baseline tryptase* | >20 | Normal or mildly increased | Normal or mildly increased | Normal |
kit D816V | + | + | – | – |
Multifocal mast cell aggregates (each with ≥15 mast cells) in the bone marrow | + | – | – | – |
Aberrant CD25 on bone marrow and other noncutaneous mast cells | + | + | – | – |
Urticaria pigmentosa¶ | +/– | – | – | – |
Mediator-release symptoms | + | + | + | + |
Hypotensive episodes | +/– | +/– | +/– | +/– |
Urine N-MH or PGD2 | Increased at baseline | Increased during symptoms | Increased during symptoms | Increased during symptoms |
Response to antimediator therapy | + | + | + | +/– |
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