Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma

Orpha code: 178522OMIM code:

Definition

A rare, primary cutaneous T-cell lymphoma characterized by solitary cutaneous nodule or only regional disease, typically occurring on the head and neck, and involving entire dermis. Sometimes, subcutis and adnexal structures are involved, as well. The infiltrate is nodular or diffuse, composed of small to medium sized pleomorphic lymphocytes and showing mild to moderate cytologic atypia. Neoplastic T-cells are mixed with B-cells, histiocytes, plasma cells and eosinophils.

Disease data
Classification

Disease

ORPHA code
178522
OMIM code
-
ICD10 code
C84.8
ICD11 code
XH7EL2

No additional description.

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