T-cell lymphoma (TCL) is a rare form of cancer that affects the lymphatic system. It develops from the abnormal multiplication of T cells, or T-lymphocytes, a type of white blood cell essential for immune system function.
The specific treatment course and prognosis for T-cell lymphoma depends on the subtype — of which there are many — and how advanced the cancer is.
There are two types of lymphoma:
Hodgkin lymphoma (formerly Hodgkin disease)
non-Hodgkin lymphoma
Hodgkin lymphoma involves a type of cancer cell called Reed-Sternberg cells. Unlike Hodgkin lymphoma, the non-Hodgkin varieties look different under the microscope and don’t contain Reed-Sternberg cells. T-cell lymphoma is a type of non-Hodgkin lymphoma.
Non-Hodgkin lymphomas either begin in B cells or T cells — T-cell lymphomas account for less than 15 percent of all non-Hodgkin lymphomas.
Types of T-Cell Lymphoma
The two main categories of T-cell lymphoma are:
Cutaneous T-cell lymphoma
Peripheral T-cell lymphoma
Typically slow-growing, cutaneous T-cell lymphoma (CTCL) is the most common form of T-cell lymphoma. It mainly affects the skin — though they’re not classified as skin cancers — but can also affect lymph nodes, blood, and internal organs.
Peripheral T-cell lymphomas are a diverse group of cancers that primarily affect lymph nodes and other lymph tissues. There are 19 different subtypes that are usually aggressive.
Given its many different subtypes, T-cell lymphoma can present with a wide range of signs and symptoms, and its manifestations may vary from person to person. For instance, the first signs of a type of T-cell lymphoma called mycosis fungoides is often a skin rash, while other forms may present with swollen lymph nodes.
Common symptoms for cutaneous T-cell lymphoma include:
Red rash that may look like a sunburn and is intensely itchy
Round scaly patches of skin that may be raised
Light or white patches of skin
Thick, raised plaques
Skin lesions, including ulcers or nodules, that may break open
Skin tumors that may be bluish or reddish brown
Changes to nails and hair, including hair loss
Thickened skin on the palms of hands and soles of feet
Skin peeling
Insomnia (from itching)
Aggressive forms of T-cell lymphoma may include symptoms like hypercalcemia (too much calcium in the blood), bone pain, and confusion.
Signs and symptoms for other types of T-cell lymphoma include:
Swollen lymph nodes, particularly in the neck, armpit, or groin
Fever or chills
Unexplained fatigue
Unexplained weight loss
Night sweats
Respiratory issues such as coughing, shortness of breath, or chest pain
Gastrointestinal symptoms such as abdominal pain, nausea, vomiting, constipation, or diarrhea
Frequent urination
Enlarged liver or spleen
Bleeding or bruising easily
Recurrent infections
If the cancer spreads to the brain, it may also cause:
Headaches
Concentration problems
Personality changes
Seizures
The causes of the majority of T-cell lymphomas are unknown. Acquired genetic mutations usually have a role to play, but external factors — including viruses and chemical exposure — may also contribute to causing T-cell lymphomas.
You have an increased risk of developing T-cell lymphoma if you:
Are age 55 or older
Are male or assigned male at birth
Are white or Asian
Have a weakened immune system due to conditions like HIV/AIDS or immunosuppressive therapies following organ transplantation
Have a family history of lymphoma or other blood cancers
Have prolonged exposure to carcinogens such as ultraviolet light, chemicals, pesticides, or environmental toxins
Have certain viral infections, such as Epstein-Barr virus and HTLV-1 (human T-lymphotropic virus 1)
Have certain autoimmune diseases, such as celiac disease
Diagnosing T-cell lymphoma involves a combination of medical and family history review, physical examination, and various diagnostic tests. During the physical examination, your healthcare provider will look for evidence of lymph nodes that can be felt through the skin, skin abnormalities, or other signs indicative of T-cell lymphoma.
Tests that may be used to diagnose T-cell lymphoma include:
Blood tests to evaluate the number and types of blood cells, and to assess organ function and overall health
Peripheral blood smear, an examination of blood cells and platelets under the microscope
CT, PET, or MRI scans to identify enlarged lymph nodes or organs affected by lymphoma and areas with abnormal cell growth
Biopsy of any enlarged lymph nodes or involved skin (to confirm lymphoma and determine its subtype)
Immunophenotyping, a lab test that helps diagnose specific lymphoma types
Bone marrow aspiration (a procedure that removes a small sample of bone marrow) and biopsy to determine if the disease has spread to the bone marrow
Flow cytometry, a test used to identify specific types of lymphoma cells
Collection and testing of cerebrospinal fluid to determine if the lymphoma has spread to the central nervous system
Tests for various viruses including HIV, HTLV-1, and Epstein-Barr virus
Your healthcare provider may also run tests to identify specific genetic mutations or molecular markers to further characterize the lymphoma and guide treatment decisions.
Accurately diagnosing T-cell lymphoma often takes time. Given that cutaneous T-cell lymphoma shares symptoms with many other benign conditions, it takes an average of 6 years from disease onset to reach a correct diagnosis.
During diagnosis, your healthcare provider will also determine the stage of your disease, which is crucial for developing an effective treatment plan.
Stages of T-Cell Lymphoma
T-cell lymphoma is typically defined as being stage 1 through 4, which indicates the extent of the cancer.
Stage 1 The cancer is limited to a single lymph node region or a single lymphoid organ, which includes the thymus, spleen, and bone marrow.
Stage 2 The cancer involves two or more lymph node regions on the same side of the diaphragm, a major breathing muscle located below the lungs.
Stage 3 The cancer involves lymph node regions on both sides of the diaphragm, above or below it, or your spleen.
Stage 4 The cancer has spread to organs beyond the lymphatic system, such as the liver, lungs, or bone marrow.
Cutaneous T-cell lymphoma has a different growth pattern than other types of lymphoma, and staging may be based on the presence of various symptoms:
Stage 1A Limited patches, papules, or plaques covering less than 10 percent of the skin surface. No tumors are present, and lymph nodes, blood, and internal organs are not involved.
Stage 1B Same as stage 1A but skin issues cover more than 10 percent of the skin surface.
Stage 2A Lymph nodes are abnormal but not cancerous.
Stage 2B One or more tumors 1 centimeter or larger develops on the skin. Lymph nodes are not cancerous.
Stage 3 At least 80 percent of the skin surface is reddened and covered with patches, papules, plaques, or tumors.
Stage 4A There is a high number of Sézary cells, or cancerous T-cells, in the blood.
Stage 4A2 There may be a high number of Sézary cells in the blood, and the lymph nodes are very abnormal or have developed cancer.
Stage 4B The cancer has spread to other organs, such as the spleen or liver.
Treatment plans are tailored to each patient, considering factors such as age, overall health, the specific characteristics of the T-cell lymphoma subtype, and stage of the disease.
Options for treatment cover a wide range of medications, procedures, and therapies.
Medication Options
Medication options may include:
Topical corticosteroids, like clobetasol propionate
Retinoids (drugs related to vitamin A), like bexarotene in the form of creams, gels, and ointments to help control redness and itchiness
Topical chemotherapy drugs like mechlorethamine
Intravenous chemotherapy drugs like doxorubicin (Adriamycin) and pralatrexate (Folotyn)
Antiviral drugs such as zidovudine (Retrovir), if there is a viral infection
Immunotherapy medications that stimulate or boost the body's immune system to recognize and attack cancer cells, including alemtuzumab (Campath, Lemtrada) and denileukin diftitox (Ontak), imiquimod (Zyclara), and pembrolizumab (Keytruda)
Targeted therapy medications that focus on specific molecular changes or proteins in or on cancer cells
Surgery
Surgery options include:
Tumor resection to remove tumors
Stem cell transplantation, a procedure to replace damaged bone marrow cells with stem cells (immature blood cells), which may require general anesthesia
Other Treatments and Procedures
Nonmedication treatment options may include:
Extracorporeal photopheresis, a procedure in which your blood is removed and treated with UV lights and light-activated drugs before being returned to your body
Radiation therapy to eliminate cancer cells, particularly when the disease is localized
Phototherapy, or using ultraviolet (UV) light to kill cancer cells in the skin
Lifestyle Approaches
While T-cell lymphoma requires thorough medical treatment, there are things you can do to encourage positive treatment outcomes.
These include:
Light exercise to maintain physical strength and reduce fatigue
A nutrient-rich diet of lean meats, fruits, and vegetables, especially cruciferous vegetables like broccoli and cauliflower
Palliative care for emotional and psychological support
Complementary therapies for symptom relief with the support of your doctor
In the case of cutaneous T-cell lymphoma:
Use mild, unscented soaps to prevent skin irritation.
Keep skin moisturized to prevent itching and infection.
Discuss bleach baths with your healthcare provider to reduce infection.
Clinical Trials
Researchers may be testing new therapies to help with conditions like T-cell lymphoma. If you're interested in participating in a clinical trial, speak with your doctor or visit ClinicalTrials.gov to search for studies in your area.
There is no definite way to prevent T-cell lymphoma. Most often, people with T-cell lymphoma have no risk factors that can be changed. But a level of awareness and certain lifestyle choices can reduce some risks, contribute to early detection, and lead to better overall prognosis. These include:
Routine health checkups and screenings to aid in the early detection of lymphoma.
Awareness of potential symptoms, and medical advice for persistent issues
A healthy weight, physical activity, and a healthy diet
Minimal exposure to environmental challenges, such as certain chemicals or pollutants
Avoidance of risk factors for HIV (which increases the risk of T-cell lymphoma) and HTLV-1, including intravenous drug use and unprotected sex
T-cell lymphoma prognosis varies widely based on the specific subtype, stage at diagnosis, and individual patient factors. Generally, T-cell lymphomas tend to have a more aggressive course than some B-cell lymphomas. Younger people, those diagnosed early, and those with localized disease that hasn't spread through the body tend to have better outcomes.
Survival rates can be influenced by:
Subtype: Different subtypes have distinct prognoses, with some being more responsive to treatment than others.
Stage at diagnosis: Early-stage T-cell lymphomas often have a more favorable prognosis than those diagnosed at an advanced stage.
Age and overall health: Younger patients and those in good overall health may respond better to treatment.
Response to treatment: How well the cancer responds to initial treatment can significantly influence the prognosis.
The overall five-year relative survival rate for non-Hodgkin lymphoma is 74.3 percent. It accounts for 3.3 percent of all cancer deaths. The survival rate for early-stage cutaneous T-cell lymphoma over 10 years is roughly 90 percent, while late stage CTCL survival rates are about 53 percent. For peripheral T-cell lymphoma, the five-year survival rate is between 30 and 40 percent.
It's crucial for you to discuss prognosis and potential treatment outcomes with your healthcare provider, as individual cases can vary widely.
T-cell lymphoma can lead to serious complications, including organ damage or life-threatening infections. Organ damage can occur when tumors penetrate areas like the spleen, liver, or bone marrow. Immune suppression or immune system reactions to tumors can lead to infections.
T-cell lymphomas are rare, making up 10 to 15 percent of all non-Hodgkin lymphomas. They are slightly more common in men or people assigned male at birth.
T-cell lymphomas occur more often in locations like Japan, China, the Caribbean, South and Central America, and West Africa, where HTLV-1 infections are more common.
Related conditions include:
Other non-Hodgkin lymphomas, like B-cell lymphoma
Skin conditions mimicking cutaneous T-cell lymphoma, like psoriasis and eczema
Autoimmune disorders like hemophagocytic syndrome
Leukemia
Some extracutaneous lymphomas, like systemic T-cell lymphoma
T-cell lymphoma may present as inflammatory skin diseases like psoriasis or dermatitis in the early stages, sometimes making diagnosis more complicated.
People with T-cell lymphoma can support each other and share effective ways to manage the disease. One of these organizations may offer the resources you need.
LLS is the largest nonprofit dedicated to curing blood cancers, including leukemia, lymphoma, Hodgkin’s disease, and myeloma. It provides patient education, offers financial assistance, and advocates for policies to improve access to care and encourage treatment development.
The Lymphoma Research Foundation is the largest U.S. nonprofit focused exclusively on lymphoma. It funds innovative research to advance treatments and cures while providing education, support services, and resources for patients and caregivers. LRF also connects people with lymphoma through its peer-to-peer Lymphoma Support Network.
CancerCare is a national nonprofit offering free professional support services for individuals affected by cancer, including counseling, support groups, educational workshops, and financial assistance.
The TCLLF supports patients with rare T-cell cancers by funding research, hosting educational forums, promoting clinical trial participation, and raising awareness about these rare diseases.
T-cell lymphoma is a rare cancer that affects the lymphatic system.
It develops from the abnormal multiplication of T cells, a type of white blood cell.
Treatment and prognosis for T-cell lymphoma depends on the subtype and how far the cancer has progressed.
The overall five-year relative survival rate for non-Hodgkin lymphomas like T-cell is 74.3 percent.
