ICD-11 classes
02 Neoplasms
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.

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:

• Mature T-cell or NK-cell neoplasms #3355
• Mature T-cell or NK-cell lymphomas and lymphoproliferative disorders, primary cutaneous specified types #3370
• Cutaneous lymphoma #17474
• Mature T-cell or NK-cell lymphomas and lymphoproliferative disorders, primary cutaneous specified types #17484