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ICD-10 CM Code D89.42

D89.42 Idiopathic mast cell activation syndrome

Excludes Type 1

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

Excludes Type 2