Background:

Monoclonal mast cell activation syndrome is included in mast cell activation disorders in which, after a diagnostic process, it is not possible to meet the required criteria for a diagnosis of systemic mastocytosis.

Clinical case:

A 73-year-old woman who presented two events of anaphylaxis 15 minutes after the intake of yucca; with a positive skin test, elevated tryptase, and mast cells with abnormal phenotype in the bone marrow biopsy, and without criteria for systemic mastocytosis.

Conclusions:

The diagnosis of monoclonal mast cell activation syndrome requires high clinical suspicion for patients with recurrent anaphylaxis and elevated tryptase, for whom joint management with hematology is essential.

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