Overview

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 and T-cell lymphoma. According to the American Cancer Society, less than 15 percent of all non-Hodgkin’s lymphomas are T-cell lymphomas.

T-cell lymphoma comes in many forms. Treatment and your general outlook depend on the type and how advanced it is at diagnosis.

What are the types of T-cell lymphoma?

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.

The two main types are of CTCL are:

  • 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 are:

  • Angioimmunoblastic lymphoma. Tends to be quite aggressive.
  • Anaplastic large cell lymphoma (ALCL). Includes three subtypes. It can affect the skin, lymph nodes, and other organs.
  • Precursor T-lymphoblastic lymphoma/leukemia. Can start in the thymus and may grow in the area between the lungs.
  • Peripheral T-cell lymphoma – unspecified. A group of diseases that don’t fit other subtypes.

Rare types include:

  • adult T-cell leukemia/lymphoma
  • extranodal natural killer/T-cell lymphoma, nasal type
  • enteropathy-associated intestinal T-cell lymphoma (EATL)
  • lymphoblastic lymphoma

What are the symptoms?

You may not have any signs of disease in the early stages. Symptoms vary according to the specific type of T-cell lymphoma.

Signs and symptoms of mycosis fungoides include:

  • patches of flat, scaly skin
  • thick, raised plaques
  • tumors that may or may not develop into ulcers
  • itching

Signs and symptoms of Sézary syndrome are:

  • red, itchy rash covering most of the body, and perhaps the eyelids
  • changes to nails and hair
  • enlarged lymph nodes
  • edema, or swelling

Not all forms of T-cell lymphoma cause symptoms on the skin. Other types may cause:

  • bleeding or bruising easily
  • recurrent infections
  • fevers or chills with no known cause
  • fatigue
  • persistent abdominal pain on the left side due to swollen spleen
  • abdominal fullness
  • frequent urination
  • constipation

Pictures of T-cell lymphoma rash

How is it treated?

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.

Skin treatments

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 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.

Systemic treatments

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 two years.

Side effects of chemotherapy can include:

  • 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

Light therapy

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

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.

Extracorporeal photopheresis

This is used to treat mycosis fungoides or Sézary syndrome. In a two-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, nausea, dizziness, and 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, and new cancers.

What are the potential complications?

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.

What’s the long-term outlook?

Overall, the five-year relative survival rate for non-Hodgkin’s lymphoma is 70 percent, according to the American Cancer Society. This is a general statistic that includes all types.

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 own doctor is in the best position to assess your situation and give you an idea of what to expect.