How Cutaneous T-cell Lymphoma is Diagnosed and Treated

Lauren Pinter-Brown, MD, Clinical Professor of Hematology/Oncology, University of California Irvine and Director of the Cutaneous Lymphoma Foundation, gives an overview of cutaneous T-cell lymphoma (CTCL) and how it is treated.

CTCL belongs to the non-Hodgkin lymphoma family as a rare group of malignancies involving 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. Pinter-Brown explains, dermatologists usually diagnose CTCL through skin biopsies. In the case of Sézary syndrome, flow cytometry of the blood may be done to diagnose the patient.

Treatment for CTCL depends on the presentation and severity of the cancer. In cases where the cancer is limited to the skin and there are no tumors, light therapy, topical nitrogen mustard, or topical retinoids are used. For more advanced stages of CTCL or aggressive subtypes like Sézary syndrome, HDAC inhibitors, chemotherapy-antibody conjugates, or interferon therapies may be used.

For more information about CTCL and other rare cancers, visit checkrare.com/diseases/cancers/