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The Spectrum of Mast Cell Activation Syndromes

Table 2. 2016 World Health Organization diagnostic criteria for systemic mastocytosis

MAJOR CRITERION 

MAJOR CRITERION

• Presence of characteristic multifocal dense mast cell infiltrates (of ≥ 15 mast cells in aggregates) in bone marrow biopsies and/or an extracutaneous organ (e.g. spleen)

• > 25% of all mast cells are atypical or spindle-shaped  

• Detection of a KIT activating point  mutation at codon 816 or in other critical regions of KIT 

• Expression of CD2 and/or CD25  and/or CD30 on the mast cells in bone marrow 

• Baseline serum tryptase is > 20 ng/mL


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