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D89.4 Mast cell activation syndrome and related disorders
BackD89Other disorders involving the immune mechanism, not elsewhere classifiedCategory7 →D89.0Polyclonal hypergammaglobulinemiaCategoryD89.1CryoglobulinemiaCategoryD89.2Hypergammaglobulinemia, unspecifiedCategoryD89.3Immune reconstitution syndromeCategoryD89.4Mast cell activation syndrome and related disordersCategory6 →D89.40Mast cell activation, unspecifiedCategoryD89.41Monoclonal mast cell activation syndromeCategoryD89.42Idiopathic mast cell activation syndromeCategoryD89.43Secondary mast cell activationCategoryD89.44Hereditary alpha tryptasemiaCategoryD89.49Other mast cell activation disorderCategoryD89.8Other specified disorders involving the immune mechanism, not elsewhere classifiedCategory5 →D89.81Graft-versus-host diseaseCategory4 →D89.810Acute graft-versus-host diseaseCategoryD89.811Chronic graft-versus-host diseaseCategoryD89.812Acute on chronic graft-versus-host diseaseCategoryD89.813Graft-versus-host disease, unspecifiedCategoryD89.82Autoimmune lymphoproliferative syndrome [ALPS]CategoryD89.83Cytokine release syndromeCategory6 →D89.831Cytokine release syndrome, grade 1CategoryD89.832Cytokine release syndrome, grade 2CategoryD89.833Cytokine release syndrome, grade 3CategoryD89.834Cytokine release syndrome, grade 4CategoryD89.835Cytokine release syndrome, grade 5CategoryD89.839Cytokine release syndrome, grade unspecifiedCategoryD89.84IgG4-related diseaseCategoryD89.89Other specified disorders involving the immune mechanism, not elsewhere classifiedCategoryD89.9Disorder involving the immune mechanism, unspecifiedCategory
Category
D89.4
Mast cell activation syndrome and related disorders
EXCLUDES
Excludes1: 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)
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)
- Chapter
- III · Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
- Section
- D80-D89
- Valid from
Source
CMS ICD-10-CM Tabular List, edition FY2026.