ICD-11 classes
03 Diseases of the blood or blood-forming organs
Neoplasms of haematopoietic or lymphoid tissues
Mature T-cell or NK-cell neoplasms
Mature T-cell or NK-cell lymphomas and lymphoproliferative...

ICD-11 Mature T-cell or NK-cell lymphomas and lymphoproliferative disorders, primary cutaneous specified types

Primary cutaneous T-cell lymphomas (CTCL) and NK-cell lymphomas are malignant lymphoproliferative diseases of unknown cause that are thought to originate from T-lymphocytes in the lymphoid tissue of the skin and by definition are confined to the skin at initial diagnosis. Included in the class is also a small number of lymphoma-like primary cutaneous lymphoproliferative disorders which are not considered to be truly malignant.

It includes 1 item.

  • Primary cutaneous peripheral T-cell lymphoma

It excludes 1 item.

  • Skin infiltration by nodal or non-cutaneous extranodal lymphoma (2E08)

Diagnosis contains 6 clarifying diagnoses:

  1. 2B00 — Subcutaneous panniculitis-like T-cell lymphoma
  2. 2B01 — Mycosis fungoides
  3. 2B02 — Sézary syndrome
  4. 2B03 — Primary cutaneous CD-30 positive T-cell lymphoproliferative disorders
    It contains 2 clarifying diagnoses.
  5. 2B0Y — Other specified primary cutaneous mature T-cell or NK-cell lymphomas and lymphoproliferative disorders
  6. 2B0Z — Primary cutaneous T-cell lymphoma of undetermined or unspecified type

The diagnosis is coded elsewhere:

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