Lymphoma is cancer that starts in the lymphocytes, a type of white blood cell of the immune system.
Lymphoma is the most common kind of blood cancer. It includes both Hodgkin’s lymphoma and non-Hodgkin’s lymphoma, depending on the specific type of lymphocyte involved.
Non-Hodgkin’s lymphoma can be divided into two groups:
- B-cell lymphoma
- T-cell lymphoma
According to the American Cancer Society, less than
T-cell lymphoma comes in many forms. Treatment and your general outlook depend on the type and how advanced it is at diagnosis.
One type of T-cell lymphoma is cutaneous T-cell lymphoma (CTCL). CTCL mainly affects the skin, but can also involve lymph nodes, blood, and internal organs.
Two main types are of CTCL
- Mycosis fungoides. This causes a variety of lesions that can easily be mistaken for other skin conditions, such as dermatitis, eczema, or psoriasis.
- Sézary syndrome. This is an advanced form of mycosis fungoides that also affects the blood. It can spread to lymph nodes and internal organs.
Other T-cell lymphomas
- Angioimmunoblastic lymphoma. It tends to be quite aggressive.
- Anaplastic large cell lymphoma (ALCL). It includes three subtypes. It can affect the skin, lymph nodes, and other organs.
- Precursor T-lymphoblastic lymphoma/leukemia. It can start in the thymus and may grow in the area between the lungs.
- Peripheral T-cell lymphoma – unspecified. This is a group of diseases that don’t fit other subtypes.
Rare types of T-cell lymphoma
- adult T-cell leukemia/lymphoma
- extranodal natural killer/T-cell lymphoma, nasal type
- enteropathy-associated intestinal T-cell lymphoma (EATL)
- lymphoblastic lymphoma
- lymphoplasmacytic lymphoma (LPL)
There are a few factors that can make you more at risk for non-Hodgkin’s lymphoma. These include:
- age (50 years and older)
- sex (more commonly found in males)
- weakened immune system
- infections, such as the ones caused by the HTLV-1 virus or Epstein-Barr virus (EBV)
You may not have any signs of the disease in the early stages. Symptoms vary according to the specific type of T-cell lymphoma.
Some signs and symptoms of mycosis fungoides
- patches of flat, scaly skin
- thick, raised plaques
- tumors that may or may not develop into ulcers
Signs and symptoms of Sézary syndrome
- red, itchy rash covering most of the body, and perhaps the eyelids
- changes to nails and hair
- enlarged lymph nodes
- edema, or swelling
Other possible signs of T-cell lymphoma
Not all forms of T-cell lymphoma cause symptoms on the skin. Here are some other things to look out for.
- bleeding or bruising easily
- recurrent infections
- fevers or chills with no known cause
- persistent abdominal pain on the left side due to swollen spleen
- abdominal fullness
- frequent urination
When diagnosing T-cell lymphoma, your doctor will likely take a biopsy from a piece of tissue that’s abnormal, such as a swollen lymph node.
The tissue will be removed and then examined through tests to determine what type of lymphoma you have.
Another way to diagnose T-cell lymphoma is through a blood test, which will measure your blood cell count. This information will help your doctor diagnose the type of lymphoma you have — particularly with mycosis fungoides and Sézary syndrome.
After determining the type of lymphoma you have, you’ll go through other tests to determine what stage it’s in. This is done through CT scans and PET scans. In cases where children have received a diagnosis of lymphoma, they may go through an MRI scan.
In terms of T-cell lymphoma, it’s rare for it to be diagnosed in advanced or later stages. Additionally, even if it has been diagnosed at an advanced stage, there are treatment options available.
Your treatment plan will depend on the type of T-cell lymphoma you have and how advanced it is. It’s not unusual to need more than one type of therapy.
Mycosis fungoides and Sézary syndrome may involve direct treatment on the skin as well as systemic treatment.
Certain ointments, creams, and gels can be applied directly to your skin to control symptoms and even destroy cancer cells. Some of these topical treatments are:
- Retinoids (vitamin A-derived drugs). Potential side effects are itching, irritation, and sensitivity to sunlight. Retinoids shouldn’t be used during pregnancy.
- Corticosteroids. Long-term use of topical corticosteroids can lead to thinning of the skin.
- Topical chemotherapy. Side effects of topical chemotherapy may include redness and swelling. It can also increase the risk of other types of cancer. However, topical chemotherapy tends to have fewer side effects than oral or intravenous chemotherapies.
Medications for T-cell lymphomas include pills, injections, and those given intravenously. Targeted therapies and chemotherapy drugs are often combined for maximum effect. Systemic treatments may include:
- a chemotherapy combination called CHOP, which includes cyclophosphamide, hydroxydoxorubicin, vincristine, and prednisone
- newer chemo drugs, such as pralatrexate (Folotyn)
- targeted drugs, such as bortezomib (Velcade), belinostat (Beleodaq), or romidepsin (Istodax)
- immunotherapy drugs, such as alemtuzumab (Campath) and denileukin diftitox (Ontak)
In advanced cases, you may need maintenance chemotherapy for up to 2 years.
Potential side effects of chemotherapy
- hair loss
- nausea and vomiting
- constipation or diarrhea
- anemia, a shortage of red blood cells, leading to fatigue, weakness, and shortness of breath
- neutropenia, a shortage of white blood cells, which can leave you vulnerable to infections
- thrombocytopenia, a shortage of blood platelets, which makes it harder for your blood to clot
UVA and UVB light can kill cancer cells on the skin. Light therapy is usually given several times a week using special lamps. UVA light treatment is combined with drugs called psoralens. UVA light activates the psoralens to kill cancer cells.
Side effects include nausea, and skin and eye sensitivity. UV light can raise the risk of developing other cancers later in life.
Radiation therapy uses radioactive particles to destroy cancer cells. The beams can be directed to the affected skin so that internal organs aren’t affected. Radiation may cause temporary skin irritation and fatigue.
This is used to treat mycosis fungoides or Sézary syndrome. In a 2-day procedure, your blood will be removed and treated with UV light and drugs that activate when exposed to the light, killing cancer cells. After the blood is treated, it’ll be returned to your body.
Side effects are minimal. However, side effects may include:
- temporary low-grade fever
- skin redness
Stem cell transplant
A stem cell transplant is when your bone marrow is replaced with marrow from a healthy donor. Prior to the procedure, you’ll need chemotherapy to suppress the cancerous bone marrow.
Complications can include:
- graft failure
- organ damage
- new cancers
If you have CTCL, skin problems may be your only symptom. Any type of cancer can eventually progress to affect lymph nodes and other internal organs.
Overall, the 5-year relative survival rate for non-Hodgkin’s lymphoma is
As with any type of cancer, it’s important to follow up with your doctor as recommended. Your recovery and outlook depend on the specific type of T-cell lymphoma and stage at diagnosis. Other considerations are type of treatment, age, and any other health conditions you might have.
Your doctor is in the best position to assess your situation and give you an idea of what to expect.