2A90.A

ICD-11 MMS 2026-01

Anaplastic large cell lymphoma, ALK-positive

Description

A T-cell peripheral lymphoma composed of usually large, pleomorphic, CD30 positive T-lymphocytes with abundant cytoplasm characterised by the presence of a translocation involving the ALK gene and expression of ALK fusion protein. Most patients present with peripheral and/or abdominal lymphadenopathy, and often have advanced disease and extranodal involvement.

Index terms

  • Anaplastic large cell lymphoma, ALK-positive
  • Anaplastic large cell lymphoma, CD30-positive

Inclusions

  • Anaplastic large cell lymphoma, CD30-positive

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
  • 2A90.3 – Aggressive NK cell leukaemia
  • 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.B – Anaplastic large cell lymphoma, ALK-negative
  • 2A90.C – Peripheral T-cell lymphoma, not otherwise specified

Foundation entity

Anaplastic large cell lymphoma, ALK-positive – http://id.who.int/icd/entity/589245621

Allowed postcoordination axes

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