2A42

ICD-11 MMS 2026-01

Juvenile myelomonocytic leukaemia

Description

A myelodysplastic/myeloproliferative neoplasm of childhood that is characterised by proliferation principally of the granulocytic and monocytic lineages. Myelomonocytic proliferation is seen in the bone marrow and the blood. The leukemic cells may infiltrate any tissue, however liver, spleen, lymph nodes, skin, and respiratory tract are the most common sites of involvement.

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

Child codes

  • 2A42.0 – Juvenile myelomonocytic leukaemia in complete remission
  • 2A42.Y – Other specified juvenile myelomonocytic leukaemia
  • 2A42.Z – Juvenile myelomonocytic leukaemia, unspecified

Sibling codes

  • 2A40 – Chronic myelomonocytic leukaemia
  • 2A41 – Atypical chronic myeloid leukaemia, BCR-ABL1-negative
  • 2A43 – Refractory anaemia with ring sideroblasts associated with marked thrombocytosis
  • 2A44 – Myeloproliferative and myelodysplastic disease, unclassifiable
  • 2A4Y – Other specified myelodysplastic and myeloproliferative neoplasms
  • 2A4Z – Myelodysplastic and myeloproliferative neoplasms, unspecified

Foundation entity

Juvenile myelomonocytic leukaemia – http://id.who.int/icd/entity/1786015803

Allowed postcoordination axes

Part of: 02: Neoplasms