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Minor diagnostic criteria include elevated serum tryptase level, abnormal MC expression of CD25 and\/or CD2, and presence of <jats:italic>KIT<\/jats:italic>D816V.<\/jats:p><jats:p><jats:bold><jats:italic>Risk stratification<\/jats:italic><\/jats:bold>: The 2008 World Health Organization classification of SM has been shown to be prognostically relevant. Classification of SM patients into indolent SM (ISM), aggressive SM (ASM), SM associated with a clonal non\u2010MC lineage disease (SM\u2010AHNMD), and mast cell leukemia (MCL) subgroups is a useful first step in establishing prognosis.<\/jats:p><jats:p><jats:bold><jats:italic>Management<\/jats:italic><\/jats:bold>: SM treatment is generally palliative. ISM patients have a normal life expectancy and receive symptom\u2010directed therapy; infrequently, cytoreductive therapy may be indicated for refractory symptoms. ASM patients have disease\u2010related organ dysfunction; interferon\u2010\u03b1 (+\/\u2212corticosteroids) can control dermatological, hematological, gastrointestinal, skeletal, and mediator\u2010release symptoms, but is hampered by poor tolerability. Similarly, cladribine has broad therapeutic activity, with particular utility when rapid MC debulking is indicated; the main toxicity is myelosuppression. Imatinib has a therapeutic role in the presence of an imatinib\u2010sensitive KIT mutation or in <jats:italic>KIT<\/jats:italic>D816\u2010unmutated patients. Treatment of SM\u2010AHNMD is governed primarily by the non\u2010MC neoplasm; hydroxyurea has modest utility in this setting; there is a role for allogeneic stem cell transplantation in select cases.<\/jats:p><jats:p><jats:bold><jats:italic>Investigational Drugs<\/jats:italic><\/jats:bold>: Recent data confirms midostaurin's significant anti\u2010MC activity in patients with advanced SM. Am. J. 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