Cutaneous T-Cell Lymphoma (CTCL) Treatment Options

Stefan M. Schieke, MD, Assistant Professor of Dermatology at University of Wisconsin-Madison, discusses how cutaneous T-cell lymphomas (CTCL) are typically treated.

There are many, multimodal treatment options for the various CTCL subtypes. In addition to pharmacologic approaches, several other types of interventions have been used successfully, including radiation therapy, phototherapy, extracorporeal photopheresis, and in some cases, stem-cell transplant. Many of these options are dependent on the stage and extent of the disease and are often categorized as “skin-directed” and systemic agents.

Patients with mycosis fungoides are often treated with skin-directed therapies, especially in the early stages of disease. In contrast, patients with later-stage disease, disease that does not respond well to skin-directed treatments, or Sézary syndrome generally require systemic treatments. Topical therapies do not have a significant impact on disease progression for advanced stages of either form of CTCL, although skin-directed treatment can play an important role in alleviating such symptoms as pain and pruritus, and most patients will require the intermittent use of topical steroids.

In early-stage CTCL, patients most often visit dermatologists for the diagnosis of their skin lesions, and typically manage it using skin-directed treatment. However, as CTCL progresses, oncologists play an important, collaborative role. The oncology team will commonly manage CTCL with systemic, targeted treatments, radiation approaches, sometimes chemotherapy for patients with Sézary syndrome, advanced disease, refractory CTCL, and other aggressive forms. Clinical trials may also be considered.
Some of the more recent directions in treatment have focused on targeted systemic therapies or targeted monoclonal antibody treatment (e.g., CCR4 or CD30). For example, brentuximab vedotin is approved for CD30-positive mycosis fungoides. Mogamulizumab, in contrast has been approved for certain MF and SS and targets specific receptors on the malignant cells (i.e. CCR4).

As Dr. Schieke 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. 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 more difficult to treat.

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.

To learn more about CTCL, visit our Cutaneous T-Cell Lymphoma (CTCL) Learning Center page: https://checkrare.com/cutaneous-t-cell-lymphoma-2/ .

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  Cutaneous T-Cell Lymphoma