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The 2016 World Health Organization (WHO) system classifies systemic mastocytosis (SM) into five categories: indolent (ISM), smoldering (SSM), SM with an associated hematological neoplasm (SM-AHN), aggressive (ASM), and mast cell leukemia (MCL). 1 WHO morphologic distinctions within SM were based on presence or absence of B (high mast cell burden, evidence of nonmast cell morphologic dysplasia or myeloproliferation, organomegaly without functional consequence) and C (cytopenia related to mast cell infiltration, palpable organomegaly with functional consequence, skeletal involvement with large osteolytic lesions, and malabsorption with weight loss secondary to gastrointestinal mast cell infiltrates) findings, presence, or absence of an associated hematological neoplasm, and presence or absence of criteria for MCL (≥ 20% mast cells on bone marrow aspirate smears or≥ 10% circulating mast cells …