Cutaneous T-Cell Lymphoma

THE INDICATION

Cutaneous T-cell lymphomas (CTCLs), also known as skin T-cell lymphomas, are cancers of the T lymphocytes which are primarily confined to the skin at diagnosis. CTCLs are a rare form of non-Hodgkin lymphomas (NHLs) and account for about 4 percent of all NHL cases.

While proper body functioning includes a balance in the body of new cells replacing old, with CTCL malignant cancer cells – known as T lymphocytes – continue to grow uncontrollably and accumulate in the skin to form patches, plaques, or tumor nodules. As the disease progresses and becomes more severe and debilitating, the malignant cells may also accumulate in the marrow, blood, lymph nodes and/or solid organs.

THE CUTANEOUS T-CELL LYMPHOMA MARKET

The annual age-adjusted incidence of CTCL is approximately six cases per one million individuals, with incidence continuing to increase over time. The average age of onset of CTCL is between 50 and 60 years, and although the disease may occur in children and young adults, incidence increases significantly with age. CTCL is twice as common in men as in women, with the highest incidence rates being reported amongst African-Americans. The two most common subtypes of CTCL are mycosis fungoides (MF), which is slow-growing in the early stages, and Sézary Syndrome (SS), which is a more aggressive form of CTCL. While research is ongoing to determine the cause of CTCL, no definitive environmental or host genetic factors have been identified to date.

Known Treatments

Specific treatments for CTCL depend on stage of the condition, ranging from less than 10 percent body surface area coverage with patches and/or plaques to any amount of skin surface covered with patches, plaques, or tumors. While patients with early-stage CTCL may respond well to skin-directed therapies, such as topical corticosteroids, chemotherapy, retinoids, and phototherapy, systemic treatments such as immune therapies, chemotherapy, and allogeneic stem cell transplantation are used when skin-directed treatments no longer benefit the patients, or in combination with skin-directed and/or other systemic agents.

While all available treatment options have benefits for patients, they are not without unwanted side-effects, making a potential new, well-tolerated, and effective treatment for CTCL a much-needed addition to the available treatment alternatives.

Source: Leukemia & Lymphoma Society, 2014, Cutaneous T-Cell Lymphoma Facts, https://www.lls.org/sites/default/-files/file_assets/cutaneoustcelllymphoma.pdf (October 2017)