D89.4Mast cell activation syndrome and related disorders
ICD-10-CM category (non-billable header) code — part of Certain disorders involving the immune mechanism (D80-D89)
Coding Notes
Excludes1 (never code both together)
- aggressive systemic mastocytosis (C96.21)
- congenital cutaneous mastocytosis (Q82.2)
- (non-congenital) cutaneous mastocytosis (D47.01)
- (indolent) systemic mastocytosis (D47.02)
- malignant mast cell neoplasm (C96.2-)
- malignant mastocytoma (C96.29)
- mast cell leukemia (C94.3-)
- mast cell sarcoma (C96.22)
- mastocytoma NOS (D47.09)
- other mast cell neoplasms of uncertain behavior (D47.09)
- systemic mastocytosis associated with a clonal hematologic non-mast cell lineage disease (SM-AHNMD) (D47.02)
Subcategories (6)
- D89.40 Mast cell activation, unspecified
- D89.41 Monoclonal mast cell activation syndrome
- D89.42 Idiopathic mast cell activation syndrome
- D89.43 Secondary mast cell activation
- D89.44 Hereditary alpha tryptasemia
- D89.49 Other mast cell activation disorder