CTCL: Mycosis Fungoides and Sézary Syndrome Overview (Chapter 1)

Joan Guitart, MD, Chief of Dermatopathology in the Department of Dermatology, Professor of Dermatology (Dermatopathology) and Pathology at Northwestern University, Feinberg School of Medicine discusses two main subtypes of cutaneous T-cell lymphoma (CTCL): mycosis fungoides and sézary syndrome.

Cutaneous T-cell lymphoma belongs to the non-Hodgkin lymphoma class of hematologic T-cell lymphoproliferative disorders. Cutaneous T-cell lymphoma is a rare group of malignancies, with an incidence of 6.4 cases per 1 million people. This form of T-cell lymphoma represents around 70% of primary cutaneous lymphomas.

The annual incidence of CTCL is 6.4 cases per 1 million people. Overall, the risk in men is twice that for women, and its likelihood increases with age. The median age at onset is in the fifth decade, but CTCL does occur (rarely) in children.

About 70% of patients with CTCL have mycosis fungoides. Its annual incidence is as high as 1 patient in 100,000 persons (the US annual incidence is about 3.6 cases per 1 million). Sézary syndrome is diagnosed in 3% to 5% of all CTCL cases.

Significant racial differences are seen in patients with mycosis fungoides. For example, the incidence of CTCL is greater in blacks and non-Hispanic whites than in other racial cohorts. Furthermore, black patients seem to present with earlier-onset and more advanced disease in comparison with white patients. In a study using the US National Cancer Database, researchers found that of 4,459 patients diagnosed with mycosis fungoides from 2004 to 2014, African-American race was an independent predictor of poorer overall survival.