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/

Stefan M. Schieke, MD, Assistant Professor of Dermatology at University of Wisconsin-Madison, discusses the role of the dermatologist in diagnosing and treating cutaneous T-cell lymphomas (CTCL).

Cutaneous T-cell lymphoma belongs to the non-Hodgkin lymphoma class of hematologic T-cell lymphoproliferative disorders. Cutaneous T-cell lymphoma is a rare group of malignancies, with an incidence of 6.4 cases per 1 million people. This form of T-cell lymphoma represents around 70% of primary cutaneous lymphomas.

Cutaneous T-cell lymphoma attacks the the body’s immune system, specifically, the lymphatic system, affecting the two types of white blood cells (lymphocytes): B-cells and T-cells. Whereas the B-lymphocytes act to neutralize the pathogens, the main job of the T-lymphocytes is to attach to these foreign cells, viruses, or cancerous growths, and directly destroy them.
Compared with other T-cell lymphomas, a distinguishing feature of CTCL is implied by the name: malignant T-cells migrating to, and collecting in, cutaneous tissue. Diagnosis can be challenging, because the initial signs and symptoms are largely skin-related and overlap with those of many other dermatologic disorders. Adding to the challenge, CTCL variants present with overlapping symptomatology, and correct identification of the CTCL subtype is key to both treatment and prognosis. Histopathologic features must be correlated with the clinical presentation to confirm the 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.

To learn more about CTCL, visit our Cutaneous T-Cell Lymphoma (CTCL) Learning Center page.

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/