Cutaneous T-cell Lymphoma Subtypes: Mycosis Fungoides and Sézary Syndrome
Anne Wood Beaven, MD, Associate Professor of Medicine and Vice Chief of Operations, Division of Hematology at University of North Carolina School of Medicine, gives an overview of cutaneous T-cell lymphoma (CTCL).
As Dr. Beaven explains, 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.
For more information about CTCL and other rare cancers, visit checkrare.com/diseases/cancers/
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