Lauren Shea, MD, Assistant Professor of Hematology and Oncology, University of Alabama, Birmingham, discusses important aspects of medical treatment and stem-cell transplantation in patients with cutaneous T-cell lymphoma (CTCL).
When faced with a patient with newly diagnosed CTCL, the oncologist generally categorizes the extent of disease by their involved “disease compartments”: skin, lymph nodes, blood, and visceral organs. Individuals with CTCL found only in the skin are considered to have early-stage disease. Those with involvement of the other compartments are classified as having advanced disease.
For those with skin-limited disease, topical steroids and light therapy (using narrow band ultraviolet light) are the best initial options. Dr. Shea indicated that it is important to work closely with dermatologists to coordinate and evaluate treatment of these patients. If these therapies produce an inadequate response, retinol-based medicines are usually the next step. The patient may require low-intensity systemic therapy if a drug like tazarotene is not effective.
For patients with advanced-stage CTCL, therapy choices depend on several factors, including whether lymph nodes, blood, visceral organs, or a combination are involved; whether the patient is CD30-positive or -negative; the patient’s symptomatology and general health status; and that person’s level of social support.
For patients with blood involvement of their CTCL, the anti-CCR4 therapy mogamulizumab can be highly effective, according to Dr. Shea. A patient who is CD30-positive may well benefit from the use of rituximab if their disease has progressed to other compartments. For those who are CD30-negative, a trial of gemcitabine may be beneficial, but a lower dose may be necessary to avoid tolerability issues.
A recent prospective investigation led to a new prognostic index for CTCL, which considers patient age, elevated lactose dehydrogenase concentrations, and cutaneous large-cell transformation. All of these may be risk factors for poorer outcomes in patients with aggressive or advanced CTCL. Patients with these risk factors may be candidates for allogeneic stem-cell transplantation, which is the only potentially curative treatment for CTCL. However, physicians must carefully consider the risk–benefit ratio of stem-cell transplant, as it is associated with a significant mortality risk. Another prospective trial did confirm improved progression-free survival and overall survival in patients with advanced CTCL undergoing stem-cell transplant versus standard therapy. “Unfortunately,” said Dr. Shea, “a majority are not cured: long-term PFS is attained by 30% to 40% of patients undergoing allotransplant.” Yet, patients’ quality of life seems better in those undergoing transplant versus standard treatment.
To learn more about CTCL and other rare cancers, visit https://checkrare.com/diseases/cancers/
