ICD-10: D89.4

Mast cell activation syndrome and related disorders

Coding Guidelines

Excludes 1

other mast cell neoplasms of uncertain behavior (D47.09)
mastocytoma NOS (D47.09)
mast cell sarcoma (C96.22)
(non-congenital) cutaneous mastocytosis (D47.01)
(indolent) systemic mastocytosis (D47.02)
congenital cutaneous mastocytosis (Q82.2)
malignant mastocytoma (C96.29)
mast cell leukemia (C94.3-)
aggressive systemic mastocytosis (C96.21)
systemic mastocytosis associated with a clonal hematologic non-mast cell lineage disease (SM-AHNMD) (D47.02)
malignant mast cell neoplasm (C96.2-)

Subcategories

D89.42

Idiopathic mast cell activation syndrome

D89.44

Hereditary alpha tryptasemia

D89.40

Mast cell activation, unspecified

D89.43

Secondary mast cell activation

D89.49

Other mast cell activation disorder

D89.41

Monoclonal mast cell activation syndrome

Related Diseases

Bamforth-Lazarus syndrome ARC syndrome rapadilino syndrome Ogden syndrome janus kinase-3 deficiency Cogan-Reese syndrome MASA syndrome CEDNIK syndrome MEDNIK syndrome respiratory allergy plum allergy 3MC syndrome 2 Yunis-Varon syndrome Miles-Carpenter syndrome Griscelli syndrome type 2

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