Cutaneous T-cell lymphomas (including rare subtypes). Current concepts. II.

Area of Science:

• Oncology
• Dermatology
• Hematology

Background:

• Cutaneous T-cell lymphomas (CTCL) constitute 80% of skin lymphomas, presenting diverse clinical and biological features.
• Recent classifications by EORTC and WHO highlight distinct primary nodal and extranodal lymphomas.
• This review focuses on T-cell and NK-cell subtypes affecting the skin.

Purpose of the Study:

• To review significant subtypes of T-cell lymphoma affecting the skin.
• To emphasize the importance of classifying CD30+ primary cutaneous lymphoproliferative disorders (PCLD).
• To discuss the clinical relevance of specific CTCL subtypes.

Main Methods:

• Review of current literature and classifications (EORTC, WHO).
• Focus on specific CTCL subtypes including mycosis fungoides, Sezary's syndrome, CD30+ PCLD, and others.
• Analysis of clinical presentation, immunophenotypic features, and outcomes.

Main Results:

• CD30+ PCLD, including lymphomatoid papulosis and CD30+ large cell lymphoma, share common features like atypical CD30+ cells and spontaneous regression.
• Most CD30+ PCLD are indolent, requiring less aggressive treatment.
• Other CTCL types include peripheral T-cell lymphoma NOS, subcutaneous panniculitis-like T-cell lymphoma, and cytotoxic lymphomas.

Conclusions:

• Accurate identification of CD30+ PCLD is crucial for patient management, as many are indolent.
• Subcutaneous panniculitis-like T-cell lymphoma may be linked to hemophagocytic syndrome.
• Cutaneous lesions in cytotoxic lymphomas can be the initial sign of systemic disease, requiring clinical vigilance.