What is the diagnosis and treatment for Mycosis fungoides (Cutaneous T-cell lymphoma)?

From the Guidelines

Mycosis fungoides should be treated with a stage-adapted conservative therapeutic approach, with skin-directed therapies such as topical steroids, PUVA, nb-UVB, or mechlorethamine being the primary treatment for early-stage disease 1. The treatment of Mycosis fungoides (Cutaneous T-cell lymphoma) depends on the stage of the disease. For patients with early-stage MF (stage IA–IIA), skin-directed therapies are recommended, including:

• Topical steroids
• PUVA
• nb-UVB
• Mechlorethamine nb-UVB can be used in patients with patches or very thin plaques, while PUVA therapy is preferred for patients with thicker plaques 1. In patients with more advanced disease, additional treatments may be necessary, such as:
• Low-dose local RT for patients with one or few infiltrated plaques or tumors (stage IIB)
• Combination therapy with PUVA and IFNa or PUVA and retinoids for patients with more extensive infiltrated plaques and tumors
• Total skin electron beam therapy (TSEBT) for patients with widespread disease
• Systemic therapies such as gemcitabine or liposomal doxorubicin for patients with advanced and refractory disease 1. It's essential to note that the treatment approach should be individualized based on the patient's specific needs and disease characteristics, and a multidisciplinary team including dermatologists and oncologists specializing in lymphoma should be involved in the management of the disease 1. For patients with advanced and refractory disease, alloSCT should be considered in relatively young patients 1. Local palliation of cutaneous and extracutaneous lesions may be achieved with local RT to doses of 8 Gy 1. The optimal conditioning regimen and timing for an allogeneic transplant are currently unknown 1.

From the FDA Drug Label

INDICATIONS AND USAGE Malignant Diseases ... The following malignancies are often susceptible to cyclophosphamide treatment: ... Mycosis fungoides (advanced disease).

The diagnosis of Mycosis fungoides (Cutaneous T-cell lymphoma) is not explicitly stated in the label, but it is mentioned as a condition that can be treated with cyclophosphamide. The treatment for Mycosis fungoides (advanced disease) is cyclophosphamide, as it is one of the malignancies often susceptible to cyclophosphamide treatment 2.

• Key points:
  • Cyclophosphamide is used to treat advanced Mycosis fungoides.
  • The label does not provide information on the diagnosis of Mycosis fungoides.

From the Research

Diagnosis of Mycosis Fungoides

• Mycosis fungoides (MF) is a type of cutaneous T-cell lymphoma that is typically diagnosed through a combination of clinical examination, biopsy, and staging [(3,4,5,6,7)].

Treatment of Mycosis Fungoides

• Treatment for MF depends on the stage of the disease and can include topical and systemic therapies [(4,5)].
• For early-stage MF, treatment options include:
  • Phototherapy (oral PUVA, NB-UVB) [(3,4,6,7)]
  • Topical corticosteroids 5
  • Topical chemotherapy 5
  • Topical retinoids 5
  • Radiotherapy 5
• For advanced-stage MF, treatment options include:
  • Systemic therapy (interferon-alpha, oral retinoids, chemotherapy) [(4,5)]
  • Combined modality therapy (total skin electron beam irradiation and chemotherapy/IFN-alpha) 4
• Novel treatments, such as retinoids (bexarotene) and immunotherapeutic agents (cytokines, tumor vaccines, and monoclonal antibodies), are also being explored 4.

Response to Treatment

• The response to treatment for MF can vary, with some patients achieving complete remission and others experiencing partial response or relapse [(3,4,7)].
• Factors that can predict disease recurrence include the presence of poikiloderma and higher disease stage 7.
• Maintenance treatment with phottherapy for at least six months may prolong remission 7.