Treatment

Consult expert advice.

Treatment options depend on the stage of the disease.

• Stage Ia-IIa
  • skin directed treatment options include topical steroids, topical chemotherapy (nitrogen mustard, carmustine), phototherapy (UVA, UVB)
• Stage IIb
  • as for Stage Ia-IIa +/- localised radiotherapy
  • as for Stage Ia-IIa +/- biological response modifiers (retinoids/interferon)
  • total skin electron beam therapy
  • single or multi-agent chemotherapy
• Stage III
  • options include:
    • extracorporeal photochemotherapy
    • single or multi-agent chemotherapy
    • as for Stage Ia-IIa +/- biological response modifiers, total skin electron beam therapy, localised radiotherapy
• Stage IV
  • options include systemic chemotherapy; radiotherapy and palliative skin directed treatment

A systematic review concluded (1):

• lack of high-certainty evidence to support decision making in the treatment of MF
• PUVA is commonly recommended as first-line treatment for MF, and we did not find evidence to challenge this recommendation
• absence of evidence to support the use of intralesional IFN-alpha or bexarotene in people receiving PUVA and an absence of evidence to support the use of acitretin or ECP for treating MF

NICE state that (2):

• chlormethine gel is recommended as an option for treating early stage (stage 1A, 1B, and 2A) mycosis fungoides-type cutaneous T-cell lymphoma (MF-CTCL) in adults

Notes (3):

• mechlorethamine (also known as chlormethine) or nitrogen mustard (NM) has a primary role in the early stages of the skin-limited disease and has been studied for its efficacy in MF since 1959
• NM is a topical chemotherapeutic alkylating agent that affects rapidly dividing cells
  • gel formulation is associated with irritation, hyperpigmentation, pruritus, erythema, and contact dermatitis

Reference:

• Valipour A et al. Interventions for mycosis fungoides. Cochrane Database of Systematic Reviews 2020, Issue 7. Art. No:CD008946. DOI: 10.1002/14651858.CD008946.pub3.
• NICE (August 2021). Chlormethine gel for treating mycosis fungoides-type cutaneous T-cell lymphoma
• Liner K, Brown C, McGirt LY. Clinical potential of mechlorethamine gel for the topical treatment of mycosis fungoides-type cutaneous T-cell lymphoma: a review on current efficacy and safety data. Drug Des Devel Ther. 2018;12:241-254.