This week LBC’s Winter-Spring Webinar series held the 3rd of our 6 lymphoma webinars, ‘T cell lymphoma’ presented by Dr Samar Issa. Non-Hodgkin lymphomas caused by T-cell lymphocytes are rare, representing about 15% of all Non Hodgkin lymphomas.

Dr Issa gives a thorough breakdown of the 4 main sub-types of T cell lymphoma, the current treatment, as well as new emerging targeted treatment. Dr Issa is Consultant haematologist and Clinical Head of the Lymphoma Service at Middlemore Hospital, Auckland. Dr Issa is an active member of a number of scientific groups; is the founding chair of the Lymphoma Network of New Zealand and the founding clinical director of the Middlemore Tissue Bank. She has multiple research publications and is the primary investigator on several local and international lymphoma clinical trials.

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Larisa Geskin, MD of Columbia University Medical Center, New York, NY discusses the future of the treatment of cutaneous T-cell lymphoma (CTCL) at the 2016 World Congress on Cancers of the Skin (WCCS) and the Congress of the European Association of Dermato-Oncology (EADO) in Vienna, Austria. According to Dr Geskin, traditional chemotherapy will give way to targeted therapies and here is a focus on genetic abnormalities and pathway abnormalities in order to find targets. In the future, CTCL will be treated with a personalized approach using antibodies or small molecules.

Brett W. shares his experience of being diagnosed with cutaneous lymphoma as a young adult.

Lymphoma Canada National Patient Conference 2022

Dr. Kevin Imrie discusses Cutaneous T-cell Lymphoma

Stefan M. Schieke, MD, Assistant Professor of Dermatology at University of Wisconsin-Madison discusses cutaneous T-cell lymphoma (CTCL) in special populations such as African Americans and veterans of war.

According to a paper published in U.S. Medicine, “The Veterans Affairs (VA) classifies CTCL and other non-Hodgkin lymphomas as presumptively caused by Agent Orange exposure or service in the Vietnam theater, even without exposure to the problematic herbicide. Both arise at markedly elevated rates in veterans, but it had remained unknown just how much higher the rate of CTCL was.”

In a study published in the Journal of Investigative Dermatology, researchers noted that “an estimated 5-15% of all CTCL diagnosed each year occurs in veterans, a percentage far greater than the percentage of veterans in the total U.S. population.” The researchers hypothesized that as the Surveillance, Epidemiology, and End Results (SEER) Program, the main source of data for national disease incidence, excludes cases from VAMCs, the incidence of CTCL in the U.S. might be underreported.1

Researchers found a 6 to 8 times higher incidence of CTCL in veterans than in the general population. The demographics and trends in the number of CTCL patients per conflict era showed great variations. Our data changes the CTCL incidence trends in the US.

The findings suggest that specific military exposures, such as Agent Orange during the Vietnam War, may be potential pathogenic drivers and warrant further exploration of the causes behind increased incidence of CTCL in veterans.

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.


References
Boyle, A: CTCL 6-10 Times More Common in Veterans; Agent Orange a Factor New Therapies Raise Optimism about Treatment. US Medicine June 12, 2018.

Del Guzzo C, Levin A, Dana A, Vinnakota R, Park Y, Newman J, Langhoff E, Geskin L. 133: The incidence of cutaneous T-cell lymphoma in the veteran population. JID. 2016May;136(5):S24.

Auris Huen, MD, PharmD, Assistant Professor, MD Anderson Cancer Center, Department of Dermatology discusses treating Cutaneous T-cell lymphoma (CTCL).

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 was recently approved for CD30-positive mycosis fungoides. Mogamulizumab, in contrast, targets specific receptors on the malignant cells (i.e., CCR4). It has been shown to improve progression-free survival, overall response rate, and duration of response in patients with mycosis fungoides and Sézary syndrome.

Stefan M. Schieke, MD, Assistant Professor of Dermatology at University of Wisconsin-Madison, provides an overview of cutaneous T-cell lymphoma (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.

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.