2A20.4

ICD-11 MMS 2026-01

Polycythaemia vera

Description

Polycythaemia vera (PV) is a clonal myeloproliferative neoplasm characterized by erythrocytosis frequently accompanied by leukocytosis, and/or thrombocytosis, and an associated increased risk of haemorrhagic and thrombotic (venous and arterial) complications. JAK2 p.V617F and JAK2 (Janus kinase 2) exon12 mutations have been strongly associated with PV.

Index terms

  • Polycythaemia vera
  • Heilmeyer-Schoner disease
  • Vaquez Osler disease
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  • Vaquez disease
  • polycythaemia rubra vera
  • primary polycythaemia

Exclusions from above levels

  • 2E03 – Malignant neoplasm metastasis in bone or bone marrow
  • http://id.who.int/icd/entity/501778925 – Malignant neoplasm metastasis in lymph nodes
  • http://id.who.int/icd/entity/97145147 – Malignant neoplasm metastasis in thymus gland

Sibling codes

  • 2A20.0 – Chronic myelogenous leukaemia, BCR-ABL1-positive
  • 2A20.1 – Chronic neutrophilic leukaemia
  • 2A20.2 – Primary myelofibrosis
  • 2A20.3 – Chronic eosinophilic leukaemia, not elsewhere classified
  • 2A20.5 – Non mast cell myeloproliferative neoplasm, unclassifiable
  • 2A20.Y – Other specified non mast cell myeloproliferative neoplasms
  • 2A20.Z – Non mast cell myeloproliferative neoplasms, unspecified

Foundation entity

Polycythaemia vera – http://id.who.int/icd/entity/818364947

Allowed postcoordination axes

Part of: 2A20: Non mast cell myeloproliferative neoplasms › 02: Neoplasms