Systemic Mastocytosis: Clinical Manifestations and Differential Diagnosis

Mast cells produce symptoms by local and remote effects of mediator release and by their presence in increased numbers in normal tissue and bone marrow, where they damage and impair normal organ function. Moreover, mast cells are long-lived and heterogeneous in their response to secretagogues and to inhibitors of mediator release. Clinicians sorting out the diagnosis of SM on the basis of presenting signs and symptoms continue to have their diagnostic skills challenged because of the rarity of this disorder, the fact that many symptoms of SM are present in more common disorders, and the multiple guises that SM may assume at the time of presentation.