2A90.3

ICD-11 MMS 2026-01

Aggressive NK cell leukaemia

Description

A rare, highly aggressive, Epstein-Barr virus-associated leukaemia, also known as aggressive NK-cell leukaemia/lymphoma; it may represent the leukemic counterpart of nasal type extranodal NK/T-cell lymphomas. It affects primarily teenagers and young adults. It is characterised by the systemic proliferation of NK cells in the peripheral blood, bone marrow, liver, and spleen.

Index terms

  • Aggressive NK cell leukaemia

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

  • 2A90.0 – T-cell prolymphocytic leukaemia
  • 2A90.1 – T-cell large granular lymphocytic leukaemia
  • 2A90.2 – Chronic lymphoproliferative disorders of NK-cells
  • 2A90.4 – Systemic Epstein-Barr Virus-positive T-cell lymphoma of childhood
  • 2A90.5 – Adult T-cell lymphoma or leukaemia, human T-cell lymphotropic virus type 1-associated
  • 2A90.6 – Extranodal NK/T-cell lymphoma, nasal type
  • 2A90.7 – Enteropathy associated T-cell lymphoma
  • 2A90.8 – Hepatosplenic T-cell lymphoma
  • 2A90.9 – Angioimmunoblastic T-cell lymphoma
  • 2A90.A – Anaplastic large cell lymphoma, ALK-positive
  • 2A90.B – Anaplastic large cell lymphoma, ALK-negative
  • 2A90.C – Peripheral T-cell lymphoma, not otherwise specified

Foundation entity

Aggressive NK cell leukaemia – http://id.who.int/icd/entity/153957345

Allowed postcoordination axes

Part of: 2A90: Mature T-cell lymphoma, specified types, nodal or systemic › 02: Neoplasms