Myeloproliferative Neoplasms – Definitions & ICD 11 Codes

Non mast cell myeloproliferative neoplasms

ICD 11 Code For Non mast cell myeloproliferative neoplasms

2A20  Non mast cell myeloproliferative neoplasms

Coded Elsewhere:

  • Acquired thrombocytosis (3B63.1)

2A20.0 Chronic myelogenous leukaemia BCR-ABL1-positive

Exclusions:

  • Atypical chronic myeloid leukaemia, BCR-ABL1- negative (2A41)
  • Chronic myelomonocytic leukaemia (2A40)
  • Other and unspecified myeloproliferative neoplasms (2A22)
  • Chronic myeloid leukaemia, not elsewhere classified (2B33.2)

2A20.00 Chronic myelogenous leukaemia with blast crisis

2A20.01 Chronic myelogenous leukaemia, Philadelphia chromosome (Ph1) positive

2A20.02 Chronic myelogenous leukaemia, t(9:22)(q34; q11)

2A20.03 Naegeli-type monocytic leukaemia

2A20.0Y Other specified chronic myelogenous leukaemia, BCR-ABL1-positive

2A20.0Z Chronic myelogenous leukaemia unspecified, BCR-ABL1-positive

2A20.1 Chronic neutrophilic leukaemia

Definition of Chronic neutrophilic leukaemia: A rare chronic myeloproliferative neoplasm characterised by sustained peripheral blood neutrophilia, bone marrow hypercellularity due to neutrophilic granulocyte proliferation, and hepatosplenomegaly. The neutrophils lack dysplasia and often show toxic granulations. There is no detectable Philadelphia chromosome or BCR/ABL1 fusion gene.

2A20.2 Primary myelofibrosis

Inclusions:

  • chronic idiopathic myelofibrosis

Exclusions:

  • Acute panmyelosis with myelofibrosis (2A60.38)

2A20.3 Chronic eosinophilic leukaemia not elsewhere classified

Definition of Chronic eosinophilic leukaemia not elsewhere classified: A chronic myeloproliferative neoplasm characterised by persistent eosinophilia in the blood, bone marrow and peripheral tissues. Organ damage occurs as a result of leukaemic infiltration or the release of cytokines, enzymes or other proteins by the eosinophils. Chronic eosinophilic leukaemia, not otherwise specified excludes patients with a Ph chromosome, BCR-ABL1 fusion gene or rearrangement of PDGFRA, PDGFRB or FGFR1.

2A20.4 Polycythaemia vera

2A20.5 Non mast cell myeloproliferative neoplasm unclassifiable

Definition of Non mast cell myeloproliferative neoplasm unclassifiable: Cases that have definite features of myeloproliferative neoplasms (MPN), but fail to meet the criteria of a specific MPN subtype.

2A20.Y Other specified non mast cell myeloproliferative neoplasms

2A20.Z Non mast cell myeloproliferative neoplasms unspecified

Mastocytosis

Definition of Mastocytosis: Mastocytosis is due to a clonal, neoplastic proliferation of mast cells that accumulate in one or more organ systems. Activating mutations of KIT are frequently found. It is characterised by the presence of multifocal compact clusters or cohesive aggregates/infiltrates of abnormal mast cells. The disorder is heterogeneous, ranging from skin lesions that may spontaneously regress to highly aggressive neoplasms associated with multiorgan failure and short survival. Subtypes of mastocytosis are recognised mainly by the distribution of the disease and clinical manifestations. In cutaneous mastocytosis (CM), the mast cell infiltration remains confined to the skin, whereas systemic mastocytosis (SM) is characterised by involvement of at least one extracutaneous organ with or without evidence of skin lesions. Mastocytosis should be strictly separated from mast cell hyperplasia or mast cell activation states without morphological and/or molecular abnormalities that characterize the neoplastic proliferation.

ICD 11 Code For Mastocytosis

  2A21  Mastocytosis

2A21.0 Systemic mastocytosis

Definition of Systemic mastocytosis: Systemic mastocytosis (SM) comprises a heterogeneous group of rare acquired and chronic haematological malignancies that are related to an abnormal proliferation of mast cells in tissue, including bone marrow, with or without skin involvement. SM can be divided into indolent SM (ISM) and aggressive SM (ASM).

2A21.00 Mast cell leukaemia

2A21.0Y Other specified systemic mastocytosis

2A21.0Z Systemic mastocytosis unspecified

2A21.1 Cutaneous mastocytosis

Definition of Cutaneous mastocytosis: Cutaneous mastocytosis is characterised by abnormal accumulation and proliferation of cutaneous mast cells. Most types are isolated but cutaneous mastocytosis can occur in association with systemic disease. Clinical forms include cutaneous mastocytoma, urticaria pigmentosa (the most frequent form), pseudoxanthomatous nodular cutaneous mastocytosis, telangiectasia macularis eruptiva perstans and diffuse cutaneous mastocytosis.

2A21.10 Urticaria pigmentosa

Inclusions:

  • Maculopapular cutaneous mastocytosis

2A21.1Y Other specified cutaneous mastocytosis

2A21.2 Mast cell sarcoma

Definition of Mast cell sarcoma: A rare entity characterised by localised but destructive growth of a tumour consisting of highly atypical, immature mast cells.

2A21.3 Extracutaneous mastocytoma

Definition of Extracutaneous mastocytoma: A localised tumour consisting of mature mast cells.

2A21.Y Other specified mastocytosis

2A21.Z Mastocytosis unspecified

Other and unspecified myeloproliferative neoplasms

ICD 11 Code For Other and unspecified myeloproliferative neoplasms

  2A22  Other and unspecified myeloproliferative neoplasms

Exclusions:

  • Chronic myelogenous leukaemia, BCR-ABL1-positive (2A20.0)
  • Atypical chronic myeloid leukaemia, BCR-ABL1- negative (2A41)

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