Anne Wood Beaven, MD, Associate Professor of Medicine and Vice Chief of Operations, Division of Hematology at University of North Carolina School of Medicine, discusses how cutaneous T-cell lymphomas (CTCL) are treated.
CTCL belongs to the non-Hodgkin lymphoma family as a rare group of malignancies. CTCL attacks the immune system, specifically, the lymphatic system which affects B-cells and T-cells. Compared to other T-cell lymphomas, CTCL involves malignant T-cells migrating to, and collecting in, cutaneous tissue. This makes diagnosis challenging as the initial signs are skin-related and, therefore, overlap with many other dermatologic disorders. Additionally, CTCL variants present overlapping symptomatology, making it difficult to diagnose between CTCL subtypes. Hence, histopathologic features must be correlated with the clinical presentation to confirm diagnosis.
Many forms of CTCL are relatively indolent compared with other T-cell lymphomas, but there are aggressive subtypes. This is illustrated by the two most common forms of CTCL: mycosis fungoides and Sézary syndrome. Although mycosis fungoides is considered a slow-growing variant, Sézary syndrome is aggressive and generally has a poor prognosis. Importantly, even the indolent subtypes can progress in some patients and become difficult to manage.
As Dr. Beaven explains, there are many treatment options for CTCL. The treatment option chosen depends on the subtype of CTCL. For relatively well-controlled subtypes, a topical cream and close surveillance by a dermatologist may be enough. For more aggressive subtypes, this is not likely to be effective. As such, for patients with mycosis fungoides or Sézary syndrome, Dr. Beaven highly encourages getting in touch with a dermatologist who specializes in or is familiar with these subtypes as they are typically very difficult to treat.
For more information about CTCL and other rare cancers, visit checkrare.com/diseases/cancers/