2025
ICD-10-CM
Diagnosis
Code

D89.4 Mast cell activation syndrome and related disorders

  • Non-billable
  • 2017–2025

D89.4 is a non-billable/non-specific diagnosis code from the 2025 ICD-10-CM used to classify the following condition: Mast cell activation syndrome and related disorders.

Type 1 excludes

  • 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)

Codes

Versions

  1. 2025

    No changes

  2. 2024

    No changes

  3. 2023

    No changes

  4. 2022

    No changes

  5. 2021

    No changes

  6. 2020

    No changes

  7. 2019

    No changes

  8. 2018

    Code changed

    • Type 1 excludes

        • aggressive systemic mastocytosis (C96.2)
        • cutaneous mastocytosis (Q82.2)
        • indolent systemic mastocytosis (D47.0)
        • malignant mastocytoma (C96.2)
        • mast cell leukemia (C94.3-)
        • mastocytoma (D47.0)
        • systemic mastocytosis associated with a clonal hematologic non-mast cell lineage disease (SM-AHNMD) (D47.0)
        • 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)
  9. 2017

    New code