2B02

ICD-11 MMS 2026-01

Sézary syndrome

Description

A generalised peripheral (mature) T-cell neoplasm characterised by the presence of erythroderma, lymphadenopathy, and neoplastic, cerebriform T-lymphocytes in the blood. Sézary syndrome is an aggressive disease.

Index terms

  • Sézary syndrome
  • Sezary syndrome

Exclusions from above levels

  • 2E08 – Skin infiltration by nodal or non-cutaneous extranodal lymphoma
  • 2E03 – Malignant neoplasm metastasis in bone or bone marrow
  • http://id.who.int/icd/entity/501778925 – Malignant neoplasm metastasis in lymph nodes
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  • http://id.who.int/icd/entity/97145147 – Malignant neoplasm metastasis in thymus gland

Sibling codes

  • 2B00 – Subcutaneous panniculitis-like T-cell lymphoma
  • 2B01 – Mycosis fungoides
  • 2B03 – Primary cutaneous CD30-positive T-cell lymphoproliferative disorders
  • 2B0Y – Other specified primary cutaneous mature T-cell or NK-cell lymphomas and lymphoproliferative disorders
  • 2B0Z – Primary cutaneous T-cell lymphoma of undetermined or unspecified type

Foundation entity

Sézary syndrome – http://id.who.int/icd/entity/1358020385

Allowed postcoordination axes

Part of: 02: Neoplasms