Sponsored by Kyowa Kirin: Understanding the Ins and Outs of CTCL


Dr. Yuliya Linhares discusses cutaneous T-cell lymphoma and the role of a multidisciplinary team of specialists in designing and managing care.

While not curable, cutaneous T-cell lymphoma (CTCL) is a rare type of blood cancer that can be a manageable disease for most patients1; however, it is key to work with a team of specialists with expertise above and below the surface of the skin when seeking care, according to Dr. Yuliya Linhares, chief of the Lymphoma, Blood and Marrow Transplant Program at Miami Center Institute.

“I would say it's extremely important for patients to find a physician who is committed and has an interest in CTCL. This disease is different in each patient and, therefore, requires an individualized approach guided by experts in dermatology and oncology,” explained Linhares.

The Basics

CTCL is a type of cancer that affects T-cells, a type of white blood cell in the body. Since it is rare and largely affects the skin, it is often mistaken for more common conditions like eczema, psoriasis or other forms of chronic dermatitis, which can delay accurate diagnosis by years or even decades.1

There are approximately 3,000 new cases of CTCL reported each year in the United States. It is more common in men and in patients older than 50 years of age, compared to younger people. In fact, by age 70, there is a four-fold increase in the number of CTCL cases.1

While each patient’s experience is unique, everyone with CTCL has skin irritation, which may include dry skin, redness, scaling patches, plaques, tumors and repeated itching and/or burning. CTCL can move beyond the skin and into the blood—and a patient may not feel or even notice it. It can also travel to lymph nodes where it can grow and affect the immune system. While rare, the internal organs may also be affected in some people.2

Most patients with CTCL will have indolent lymphoma, which means it is chronic and slow growing. Therefore, while not curable, the disease is highly treatable at all stages and usually is not life-threatening.1

There are two main subtypes of CTCL which most people are classified into: mycosis fungoides and Sézary syndrome.1 Knowing the subtype can help patients understand their CTCL.

Mycosis Fungoides and Sézary Syndrome

Mycosis fungoides is the most common form of CTCL (50-70%), with some progressing to advanced-stage disease (~12%).3,4 It is more common in men, in Blacks compared to whites, and in patients older than 50 years of age.5

One of the biggest challenges associated with the diagnosis of mycosis fungoides is that it doesn’t look the same in all patients. It can appear anywhere on the body, but tends to affect areas of the skin protected from the sun by clothing. Lesions can appear as patches, which are usually flat, can be smooth or scaly, and look like a rash; as plaques, which are thicker, raised, usually scaly lesions; or as tumors, which are raised bumps or nodules that may or may not ulcerate.5

In early stages, many people only experience skin symptoms, which may include redness, rashes and smaller patches. But if these symptoms progress, larger areas of skin will be affected with more intense redness, scaling, and lesions. In some people, mycosis fungoides can progress to lymph nodes and internal organs over many years, often decades.3

Unlike mycosis fungoides, Sézary syndrome is not as common, affecting less than 5% of those with the disease.6 It is most common in people 50 years of age or older, and is slightly more common in men. It is a leukemic form of CTCL, meaning cancerous white blood cells are present in the blood. The most noticeable symptom is an extensive red, itchy rash covering at least 80% of the body.7 Sometimes, it can spread to the lymph nodes and internal organs. Since Sézary syndrome can be very serious, it usually requires more aggressive approaches than mycosis fungoides.7

Diagnosing and Tracking CTCL

Since CTCL is a chronic condition that will be managed over time, it’s important to have the right care team to help guide treatment and track progress.

“Having a multidisciplinary team of experts involved from the beginning can aid in the proper diagnose and staging of CTCL and in the development of a treatment plan that meets each patient’s needs,” Linhares explained.

“Dermatologists are typically most familiar with the earliest stage of the disease when there is only skin involvement. Because CTCL is a blood cancer, it's also good to have an oncologist and a radiation oncologist involved from the beginning to help monitor progression and to make sure that the appropriate treatments are initiated in a timely manner and in the right sequence,” she added.

As part of a CTCL diagnosis, members of the care team will typically conduct a physical exam and ask certain questions to understand how itchy the skin may be, and how it may be affecting a person’s life. A skin biopsy may be taken and blood tests performed to confirm diagnoses and determine the subtype and staging of the disease. Organs may also be checked, including the lymph nodes, spleen, liver, and lungs. Beyond diagnosis, these tests can assist in tracking disease progression as well. It is essential that any diagnosis be confirmed by a pathologist who has expertise in diagnosing cutaneous lymphomas.5,7

While treatment and monitoring should be coordinated by the care team, it’s still important for patients to be their own advocates. Dr. Linhares recommends keeping a journal that captures test results, treatment taken, and how symptoms change over time; pictures of skin patches, plaques and tumors should be included, if possible. This log should be shared as part of care team appointments to provide a clear picture of the patient’s experience and how the disease may be improving or progressing.

Treatment Options

A number of factors go into treatment decisions for CTCL – such as the extent of skin involvement, the type of skin lesion, and whether the cancer has spread to the blood, lymph nodes or other internal organs – and more, treatments can vary from patient to patient, depending on symptoms, disease stage and a patient’s health status.3

Treatments are either directed at the skin or the entire body. For example, topical treatments are applied directly to the skin are the first-line of treatment, and can be used in patients with mycosis fungoides.5 Phototherapy or use of ultraviolet light to decrease inflammation in the skin has also been proven as an effective treatment.3 Meanwhile, some patients will also need a systemic treatment that is either taken by mouth, injected or infused so that the treatment can spread throughout the body.5

Because treatment varies by the patient, it is important for individuals to work with their care teams to determine the treatment option that is best for them and will address their needs.

For more information, visit https://www.insidectcl.com.


  1. Cutaneous T-Cell Lymphoma. Cutaneous Lymphoma Foundation. https://www.clfoundation.org/cutaneous-t-cell-lymphoma.
  2. A Patient’s Guide to Understanding Cutaneous Lymphoma. Cutaneous Lymphoma Foundation. https://www.clfoundation.org/sites/default/files/2017-08/2013_guide.pdf
  3. Cutaneous T-Cell Lymphoma. Leukemia & Lymphoma Society. https://www.lls.org/sites/default/files/file_assets/PS96_CTCL_Booklet_Final.pdf.
  4. Talpur R, Singh L, Daulat S, et al. Long-term Outcomes of 1,263 Patients with Mycosis Fungoides and Sezary Syndrome from 1982 to 2009. Clin Can Res. 2012;18(18):5051-5060. doi:10.1158/1078-0432.ccr-12-0604.
  5. Mycosis Fungoides. Cutaneous Lymphoma Foundation. https://www.clfoundation.org/mycosis-fungoides
  6. National Cancer Comprehensive Network. Primary Cutaneous Lymphomas. NCCN Clinical Practice Guidelines in Oncology. 2020;2.2020:1-98.
  7. Sézary syndrome. Cutaneous Lymphoma Foundation. https://www.clfoundation.org/sezary-syndrome