Current approaches to the diagnosis and treatment of systemic mastocytosis

Mastocytosis should be suspected in patients who present with a constellation of symptoms, including flushing, abdominal pain, diarrhea, unexplained syncope, and classic urticaria pigmentosa lesions. Diagnosis should be established by a bone marrow biopsy in all adults. Staging should be performed to assess disease burden and evidence of end-stage organ damage. Patients should be offered symptom-based treatment and cytoreductive therapy only for aggressive systemic mastocytosis or an associated hematologic malignant neoplasm.