2021

  1. ICD-10-CM Codes
    2. ›
  2. D50-D89 Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
    3. ›
  3. D80-D89 Certain disorders involving the immune mechanism
    4. ›
  4. D89- Other disorders involving the immune mechanism, not elsewhere classified
    5. ›
  5. 2021 ICD-10-CM Diagnosis Code D89.4

2021 ICD-10-CM Diagnosis Code D89.4

Mast cell activation syndrome and related disorders

    2017 - New Code 2018 2019 2020 2021 Non-Billable/Non-Specific Code
  • D89.4 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail.
  • The 2021 edition of ICD-10-CM D89.4 became effective on October 1, 2020.
  • This is the American ICD-10-CM version of D89.4 - other international versions of ICD-10 D89.4 may differ.
Type 1 Excludes
Type 1 Excludes Help
A type 1 excludes note is a pure excludes. It means "not coded here". A type 1 excludes note indicates that the code excluded should never be used at the same time as D89.4. A type 1 excludes note is for used for when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • aggressive systemic mastocytosis (
    ICD-10-CM Diagnosis Code C96.21

    Aggressive systemic mastocytosis

      2018 - New Code 2019 2020 2021 Billable/Specific Code
    C96.21
    )
  • congenital cutaneous mastocytosis (
    ICD-10-CM Diagnosis Code Q82.2

    Congenital cutaneous mastocytosis

      2016 2017 2018 - Revised Code 2019 2020 2021 Billable/Specific Code POA Exempt
    Applicable To
    • Congenital diffuse cutaneous mastocytosis
    • Congenital maculopapular cutaneous mastocytosis
    • Congenital urticaria pigmentosa
    Type 1 Excludes
    • cutaneous mastocytosis NOS (D47.01)
    • diffuse cutaneous mastocytosis (with onset after newborn period) (D47.01)
    • malignant mastocytosis (C96.2-)
    • systemic mastocytosis (D47.02)
    • urticaria pigmentosa (non-congenital) (with onset after newborn period) (D47.01)
    Q82.2
    )
  • (non-congenital) cutaneous mastocytosis (
    ICD-10-CM Diagnosis Code D47.01

    Cutaneous mastocytosis

      2018 - New Code 2019 2020 2021 Billable/Specific Code
    Applicable To
    • Diffuse cutaneous mastocytosis
    • Maculopapular cutaneous mastocytosis
    • Solitary mastocytoma
    • Telangiectasia macularis eruptiva perstans
    • Urticaria pigmentosa
    Type 1 Excludes
    • congenital (diffuse) (maculopapular) cutaneous mastocytosis (Q82.2)
    • congenital urticaria pigmentosa (Q82.2)
    • extracutaneous mastocytoma (D47.09)
    D47.01
    )
  • (indolent) systemic mastocytosis (
    ICD-10-CM Diagnosis Code D47.02

    Systemic mastocytosis

      2018 - New Code 2019 2020 2021 Billable/Specific Code
    Applicable To
    • Indolent systemic mastocytosis
    • Isolated bone marrow mastocytosis
    • Smoldering systemic mastocytosis
    • Systemic mastocytosis, with an associated hematological non-mast cell lineage disease (SM-AHNMD)
    Code Also
    • , if applicable, any associated hematological non-mast cell lineage disease, such as:
    • acute myeloid leukemia (C92.6-, C92.A-)
    • chronic myelomonocytic leukemia (C93.1-)
    • essential thrombocytosis (D47.3)
    • hypereosinophilic syndrome (D72.1)
    • myelodysplastic syndrome (D46.9)
    • myeloproliferative syndrome (D47.1)
    • non-Hodgkin lymphoma (C82-C85)
    • plasma cell myeloma (C90.0-)
    • polycythemia vera (D45)
    Type 1 Excludes
    • aggressive systemic mastocytosis (C96.21)
    • mast cell leukemia (C94.3-)
    D47.02
    )
  • malignant mast cell neoplasm (
    ICD-10-CM Diagnosis Code C96.2

    Malignant mast cell neoplasm

      2016 2017 2018 - Converted to Parent Code 2019 2020 2021 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • indolent mastocytosis (D47.02)
    • mast cell leukemia (C94.30)
    • mastocytosis (congenital) (cutaneous) (Q82.2)
    C96.2-
    )
  • malignant mastocytoma (
    ICD-10-CM Diagnosis Code C96.29

    Other malignant mast cell neoplasm

      2018 - New Code 2019 2020 2021 Billable/Specific Code
    C96.29
    )
  • mast cell leukemia (
    ICD-10-CM Diagnosis Code C94.3

    Mast cell leukemia

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    C94.3-
    )
  • mast cell sarcoma (
    ICD-10-CM Diagnosis Code C96.22

    Mast cell sarcoma

      2018 - New Code 2019 2020 2021 Billable/Specific Code
    C96.22
    )
  • mastocytoma NOS (
    ICD-10-CM Diagnosis Code D47.09

    Other mast cell neoplasms of uncertain behavior

      2018 - New Code 2019 2020 2021 Billable/Specific Code
    Applicable To
    • Extracutaneous mastocytoma
    • Mast cell tumor NOS
    • Mastocytoma NOS
    • Mastocytosis NOS
    D47.09
    )
  • other mast cell neoplasms of uncertain behavior (
    ICD-10-CM Diagnosis Code D47.09

    Other mast cell neoplasms of uncertain behavior

      2018 - New Code 2019 2020 2021 Billable/Specific Code
    Applicable To
    • Extracutaneous mastocytoma
    • Mast cell tumor NOS
    • Mastocytoma NOS
    • Mastocytosis NOS
    D47.09
    )
  • systemic mastocytosis associated with a clonal hematologic non-mast cell lineage disease (SM-AHNMD) (
    ICD-10-CM Diagnosis Code D47.02

    Systemic mastocytosis

      2018 - New Code 2019 2020 2021 Billable/Specific Code
    Applicable To
    • Indolent systemic mastocytosis
    • Isolated bone marrow mastocytosis
    • Smoldering systemic mastocytosis
    • Systemic mastocytosis, with an associated hematological non-mast cell lineage disease (SM-AHNMD)
    Code Also
    • , if applicable, any associated hematological non-mast cell lineage disease, such as:
    • acute myeloid leukemia (C92.6-, C92.A-)
    • chronic myelomonocytic leukemia (C93.1-)
    • essential thrombocytosis (D47.3)
    • hypereosinophilic syndrome (D72.1)
    • myelodysplastic syndrome (D46.9)
    • myeloproliferative syndrome (D47.1)
    • non-Hodgkin lymphoma (C82-C85)
    • plasma cell myeloma (C90.0-)
    • polycythemia vera (D45)
    Type 1 Excludes
    • aggressive systemic mastocytosis (C96.21)
    • mast cell leukemia (C94.3-)
    D47.02
    )
The following code(s) above D89.4 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to D89.4:
  • D50-D89
    2021 ICD-10-CM Range D50-D89

    Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism

    Type 2 Excludes
    • autoimmune disease (systemic) NOS (M35.9)
    • certain conditions originating in the perinatal period (P00-P96)
    • complications of pregnancy, childbirth and the puerperium (O00-O9A)
    • congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
    • endocrine, nutritional and metabolic diseases (E00-E88)
    • human immunodeficiency virus [HIV] disease (B20)
    • injury, poisoning and certain other consequences of external causes (S00-T88)
    • neoplasms (C00-D49)
    • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)
    Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
  • D80-D89
    2021 ICD-10-CM Range D80-D89

    Certain disorders involving the immune mechanism

    Includes
    • defects in the complement system
    • immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
    • sarcoidosis
    Type 1 Excludes
    • autoimmune disease (systemic) NOS (M35.9)
    • functional disorders of polymorphonuclear neutrophils (D71)
    • human immunodeficiency virus [HIV] disease (B20)
    Certain disorders involving the immune mechanism
  • D89
    ICD-10-CM Diagnosis Code D89

    Other disorders involving the immune mechanism, not elsewhere classified

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • hyperglobulinemia NOS (R77.1)
    • monoclonal gammopathy (of undetermined significance) (D47.2)
    Type 2 Excludes
    • transplant failure and rejection (T86.-)
    Other disorders involving the immune mechanism, not elsewhere classified
Code History
  • 2017 (effective 10/1/2016): New code
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
  • 2021 (effective 10/1/2020): No change
ICD-10-CM Codes Adjacent To D89.4
D86.85 Sarcoid myocarditis
D86.86 Sarcoid arthropathy
D86.87 Sarcoid myositis
D86.89 Sarcoidosis of other sites
D86.9 Sarcoidosis, unspecified
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
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
D89.49 Other mast cell activation disorder
D89.8 Other specified disorders involving the immune mechanism, not elsewhere classified
D89.81 Graft-versus-host disease
D89.810 Acute graft-versus-host disease
D89.811 Chronic graft-versus-host disease
D89.812 Acute on chronic graft-versus-host disease

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.

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