Henry Wong, MD, PhD, Dermatologist at the San Diego Veterans Hospital and the University of California San Diego (UCSD), discusses cutaneous T-cell lymphoma (CTCL).
CTCL is a rare group of malignancies that attack the body’s immune lymphatic system, affecting both B-cells and T-cells. The underlying cause of CTCL is not fully understood. Genetic causes are suspected but these issues appear to be sporadic mutations for the most common forms of CTCL. The most common subtypes of CTCL are Sézary syndrome and mycosis fungoides.
Symptoms and Diagnosis
Common symptoms include widespread red rash that may cover most of the body, the presence of cancerous T cells in the blood, and abnormally enlarged lymph nodes. Other signs and symptoms may include intense itchiness, scaling and peeling of the skin; fever; weight loss; hair loss; ectropion; palmoplantar keratoderma; malformation of the nails; and hepatosplenomegaly.
The prevalence of the disease is around 3,000 new cases per year in the United States. However, due to its indolent nature, many are not aware of their disease right away. Additionally, diagnosis can be difficult due to symptoms that present as less rare skin conditions such as atopic dermatitis or psoriasis. Dr. Wong notes the unmet need for better diagnostic markers that differentiate between CTCL and less-rare conditions as well as between subtypes of CTCL.
Treatment
Dr. Wong highlights the importance of multidisciplinary treatment for patients with CTCL. This is crucial due to the patient’s various stages of disease and the heterogeneity of symptoms. While dermatologists can provide skin directed treatment and phototherapy, some patients experience poor response to topical first-line treatment and may require more systemic treatment that can be accessed via collaboration with oncologists and radiologists.
Due to slow progression of disease and its effect on the immune system, chemotherapy is not viewed as the best treatment for CTCL. Instead, clinicians tend to prefer targeted therapies that address specific disease involvement more effectively. Combination treatments tailored to patients’ unique disease stage, symptoms, and T-cell markers are typically the most effective and lead to less side effect burden.
To learn more about CTCL, visit our CTCL Learning Page.