D89.4 Mast cell activation syndrome and related disorders

Non-billable header with 6 child codes.

Excludes1 (never code 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)

Applies from D89 Other disorders involving the immune mechanism, not elsewhere classified

Excludes1 (never code together)

• hyperglobulinemia NOS (R77.1)
• monoclonal gammopathy (of undetermined significance) (D47.2)

Excludes2 (not included here)

• transplant failure and rejection (T86.-)

Codes under D89.4

• 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

Code annotations containing back-references to D89.4

Back-references to D89

• Type 1 Excludes: D72 Other disorders of white blood cells ("immunity disorders (D80-D89)")

Nearby codes

• D89 Other disorders involving the immune mechanism, not elsewhere classified
• D89.0 Polyclonal hypergammaglobulinemia
• D89.1 Cryoglobulinemia
• D89.2 Hypergammaglobulinemia, unspecified
• D89.3 Immune reconstitution syndrome
• D89.4 Mast cell activation syndrome and related disorders (this code)
• 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