Cutaneous T-Cell Lymphoma, Mycosis Fungoides
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Cutaneous T-cell lymphoma (CTCL) is a rare and typically indolent mature T-cell lymphoma presenting primarily in the skin. This disease involves overlap of the disciplines of dermatology, medical oncology, and radiation oncology.
- A heterogeneous group of relatively uncommon extranodal non-Hodgkin lymphomas
- This topic focuses on mycosis fungoides (MF), the most common subtype of cutaneous lymphoma. Other subtypes include Sézary syndrome and primary cutaneous anaplastic large cell lymphoma (ALCL). For other subtypes, please consult the Reference section.
- Median age at diagnosis is 55 to 60 years; however, it can occur in children and young adults.
- Male-to-female ratio = 2:1
- Incidence is higher in African Americans.
- 0.6 cases per 100,000 per year
- ~4% of all non-Hodgkin lymphoma cases
Etiology and Pathophysiology
- Unknown but thought to be due to genetic and epigenetic abnormalities
- Infiltration of activated and malignant T cells in the skin
- Cytokines, such as interleukin (IL)-4 and IL-5, which can lead to eosinophilia and atopy-like symptoms
- Clonal T-cell receptor (TCR) gene rearrangements are detected in most cases.
- No recurrent, MF-specific chromosomal translocations have been identified.
- Loss at chromosome 10q and abnormalities in the tumor suppressor genes p15, p16, and p53 are common.
- Epigenetic changes may play an important role (1)[A].
Possible risk factors include viral infection (HTLV-1, EBV) or solvent/chemical exposure.