Overview:
- CTCL is a group of non-Hodgkin T-cell lymphomas that primarily affect the skin.
- Most common subtype: Mycosis fungoides (MF)
- Advanced variant: Sézary syndrome (leukemic form of CTCL)
Pathophysiology:
- Malignant proliferation of mature, skin-homing CD4+ T cells
- Indolent course initially, may progress to systemic involvement
Clinical Presentation:
- Early-stage: Eczema-like patches or plaques (often misdiagnosed)
- Advanced-stage: Tumors, lymphadenopathy, blood and visceral involvement
- Sézary syndrome: Diffuse erythroderma, lymphadenopathy, circulating malignant T-cells (Sézary cells)
Diagnosis:
- Skin biopsy: Histopathology and T-cell clonality
- Immunophenotyping: CD3+, CD4+, loss of CD7/CD26
- Staging: Skin (T), nodes (N), blood (B), viscera (M) – TNMB classification
Treatment (Stage-dependent):
Early-Stage (IA–IIA):
- Skin-directed therapy:
- Topical corticosteroids
- Topical chemotherapy (e.g., mechlorethamine gel)
- Phototherapy (PUVA, UVB)
- Localized radiation
Advanced-Stage (≥IIB or Sézary):
- Systemic therapy:
- Retinoids: Bexarotene (oral)
- HDAC inhibitors: Vorinostat, romidepsin
- Monoclonal antibodies:
- Brentuximab vedotin (CD30+ disease)
- Mogamulizumab (CCR4+) for relapsed/refractory cases
- Interferon-alpha
- Methotrexate (low-dose)
- ECP (extracorporeal photopheresis) – especially in Sézary
- Chemotherapy: Reserved for refractory cases (e.g., CHOP)
- Allogeneic stem cell transplant: in select advanced/refractory cases
Pharmacist Considerations:
- Monitor for photosensitivity with phototherapy and retinoids
- Thyroid and lipid monitoring with bexarotene
- QT prolongation risk with HDAC inhibitors (ECG, electrolytes)
- Infusion reactions with monoclonal antibodies (premedications needed)
- Monitor skin infections, cytopenias, and disease flares
- Screen for hepatitis B before anti-CD20/CD30 therapy (if used)
Synonyms
CTCL