Treating T-Cell Non-Hodgkin Lymphoma

Cutaneous T-cell lymphomas

Includes mycosis fungoides, Sézary syndrome, and other skin lymphomas.

Cutaneous T-cell lymphomas start in the skin. The most common types are mycosis fungoides and Sézary syndrome.

To learn more, see Treating Lymphoma of the Skin.

Peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS)

This name is given to many T-cell lymphomas that don't fit well into any of the other groups listed below. Most T-cell lymphomas are classified as PTCL, NOS.

These lymphomas usually grow quickly. They can start in the lymph nodes, but they often involve other organs, including the skin and bone marrow.

Initial treatment

PTCL, NOS is usually treated with intense chemotherapy (chemo) using a combination of drugs, such as:

• CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone)
• CHOEP (CHOP plus etoposide)

If the lymphoma cells have the CD30 protein, another option is CHP (cyclophosphamide, doxorubicin, and prednisone) along with the antibody-drug conjugate brentuximab vedotin (Adcetris).

Radiation therapy might be used if the lymphoma is in no more than a few areas.

Stem cell transplant

This lymphoma often comes back after chemo. So if it responds well to treatment and you are healthy enough, doctors may suggest a stem cell transplant using your own stem cells.

If the lymphoma doesn’t respond or comes back

If the lymphoma doesn’t respond or comes back after treatment, other drugs might help. These include:

• Pralatrexate (Folotyn)
• Belinostat (Beleodaq)
• Brentuximab vedotin (if the lymphoma cells have the CD30 protein)

Some people may be able to have a stem cell transplant using cells from a donor. Because this lymphoma can be hard to treat, a clinical trial may also be a good option.

Angioimmunoblastic T-cell lymphoma (AITL)

AITL is a fast-growing lymphoma. It often causes fevers, rashes, immune system problems, and swollen lymph nodes.

In the newest classification systems, AITL is grouped with a few closely related lymphomas called T-follicular helper (TFH) cell lymphomas.

Initial treatment

AITL is typically treated with intense chemo, using a combination of drugs, such as:

• CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone)
• CHOEP (CHOP plus etoposide)

If the lymphoma cells have the CD30 protein, another option might be CHP (cyclophosphamide, doxorubicin, and prednisone) along with the antibody-drug conjugate brentuximab vedotin (Adcetris).

For people who can’t handle intense chemo, a single chemo or immunotherapy drug might be an option.

Radiation therapy may be an option in rare cases, when the lymphoma is only in one area.

Stem cell transplant

Standard doses of chemo often don’t keep this lymphoma from coming back. Doctors often suggest a stem cell transplant after initial chemotherapy, if a person can tolerate it.

Anaplastic large cell lymphoma (ALCL)

ALCL is a fast-growing lymphoma that mainly affects lymph nodes.

Treatment typically includes intense chemotherapy (chemo) using a combination of several drugs. This is sometimes given along with radiation therapy if the lymphoma is confined to no more than a few areas.

This type of lymphoma often responds well to treatment, especially if the lymphoma cells have too much of the ALK protein.

Initial treatment

A common initial treatment is CHP (cyclophosphamide, doxorubicin, and prednisone) along with the antibody-drug conjugate brentuximab vedotin (Adcetris).

Other options include CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) or CHOEP (CHOP plus etoposide).

If the lymphoma comes back

If the lymphoma returns after initial treatment, options include:

• Brentuximab vedotin alone
• Crizotinib (Xalkori), a targeted drug for ALK-positive ALCL in children and young adults
• Other drugs, such as pralatrexate (Folotyn) or belinostat (Beleodaq)
• Other chemo combinations

To some extent, your options will depend on whether or not you plan to get a stem cell transplant eventually.

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL)

This rare type of ALCL is linked to breast implants with a textured (rough) surface. It usually shows up as fluid or a lump near the implant, often many years after the implant was placed.

If ALCL develops in the capsule (scar tissue) that forms around a breast implant, doctors typically remove the implant and the capsule surrounding it in one piece, along with any lumps. There is a risk of lymphoma in the other breast, so the implant on the other side is usually removed as well.

• If the lymphoma is only in the capsule, surgery alone is usually enough treatment.
• If the lymphoma cannot be completely removed completely with surgery, you might also get radiation therapy to the area.
• If the lymphoma has spread, additional treatment might include chemo. This is usually with one of the same regimens listed above for ALCL, sometimes along with radiation.

Adult T-cell leukemia/lymphoma (ATLL)

ATLL is caused by infection with the HTLV-1 virus. ATLL is rare in the United States. It is seen more often in Japan, the Caribbean, and parts of South America and Africa, where infection with HTLV-1 is more common.

Treatment depends on which subtype you have.

Smoldering or chronic subtypes

Smoldering or chronic subtypes of ATLL usually grow slowly. If the lymphoma isn’t causing problems other than mildly swollen lymph nodes, it can often be watched without treating it right away.

If treatment is needed:

• One option is interferon and the antiviral drug zidovudine to fight the HTLV-1 infection.
• If the lymphoma is affecting the skin, it may be treated with radiation or other treatments aimed at the skin.
• Another option might be chemo or other combinations. For more on this, see Common chemo regimens for ATLL(below).

Some chronic ATLL is more likely to grow quickly. If so, it is usually treated like the faster-growing subtypes.

Acute and lymphoma subtypes

The acute and lymphoma subtypes of ATLL grow quickly and can be hard to treat. 

• The acute subtype can be treated with either antiviral drugs or chemo. For more on this, see Common chemo regimens for ATLL (below).
• The lymphoma subtype is typically treated with chemo. Antiviral therapy is not helpful.

The acute and lymphoma subtypes can both spread to the tissues around the brain and spinal cord, so chemo is often given into the spinal fluid as well. This is called intrathecal chemo.

If the disease responds well to treatment, a stem cell transplant using cells from a donor might be an option. This type of transplant offers the best chance of keeping the lymphoma from coming back. However, not everyone is healthy enough to have one.

Common chemo regimens for ATLL

Common chemo regimens for ATLL include:

• EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin)
• CHP (cyclophosphamide, doxorubicin, and prednisone) plus the antibody-drug conjugate brentuximab vedotin (Adcetris), if the lymphoma cells have the CD30 protein
• Hyper-CVAD (cyclophosphamide, vincristine, doxorubicin, and dexamethasone) alternating with high-dose methotrexate and cytarabine (ara-C)

If these treatments aren’t effective or if the lymphoma comes back, other chemo or immunotherapy drugs might still be helpful.

There is no clear standard treatment for this disease, so enrolling in a clinical trial might be a good option if one is available.

Intestinal T-cell lymphomas

Several types of T-cell lymphoma may start in the intestines (bowels). One kind, called enteropathy-associated intestinal T-cell lymphoma, is strongly linked to celiac disease.

Intensive chemo

Intensive chemo using several drugs is usually the main treatment. Common drug combinations include:

• CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone)
• CHOEP (CHOP plus etoposide)

If the lymphoma cells have the CD30 protein, another option might be the CHP chemo regimen (cyclophosphamide, doxorubicin, and prednisone) along with the antibody-drug conjugate brentuximab vedotin (Adcetris).

Other treatments

• For people who can’t tolerate intense chemo, a single chemo or immunotherapy drug might be an option.
• If the lymphoma is only in one area, radiation therapy may be an option.
• A stem cell transplant may be an option if the lymphoma responds to chemo.

Surgery

If these treatments work, one concern is that a hole (perforation) might develop in the intestines as the lymphoma cells die. You might first have surgery to remove the part of the intestines containing the lymphoma.

If the lymphoma caused a perforation or intestinal blockage (obstruction) before you were diagnosed, you may also need surgery before chemo or radiation.

Extranodal natural killer (NK)/T-cell lymphoma, nasal type

This rare lymphoma is linked to infection with the Epstein-Barr virus (EBV). It is more common in parts of Asia and Central and South America. It most often starts in the nasal passages or upper throat, but it can also affect other parts of the body, such as the skin or intestines.

Initial treatment

• This lymphoma is usually treated with chemo that includes a drug called asparaginase (or pegaspargase).
• Some chemo combinations commonly used for other lymphomas, such as CHOP, don’t work as well for this type.

If the lymphoma doesn’t go away or comes back

If the lymphoma doesn’t go away completely, a stem cell transplant may be done if possible.

If the lymphoma comes back after treatment, other chemo drugs may be tried. Immunotherapy drugs called checkpoint inhibitors can also help some people. Because this lymphoma can be hard to treat when it comes back, a clinical trial may be a good option.

T-lymphoblastic lymphoma/leukemia

This type of lymphoma is also known as T-cell acute lymphoblastic leukemia/lymphoma. Doctors may call this a lymphoma or a leukemia, depending on the amount of cancer cells in the bone marrow. But both grow quickly and are treated the same way.

Although this lymphoma is fast-growing, it can often be cured with treatment, especially in children and young adults. Treatment is usually similar to the treatment for acute lymphocytic leukemia (ALL).

Initial treatment

This fast-growing disease needs to be treated with intensive chemotherapy (chemo), when possible. Treatment is typically given in the hospital at first.

Chemo: Combinations of many chemo drugs are used. These can include cyclophosphamide, daunorubicin or doxorubicin (Adriamycin), vincristine, L-asparaginase (or related drugs), methotrexate, prednisone or dexamethasone, and sometimes cytarabine (ara-C).

Intrathecal chemo: Because of the risk of spread to the brain and spinal cord, a chemo drug such as methotrexate is also given into the spinal fluid.

Preventing tumor lysis syndrome: During the initial treatment phase, you will be at risk for tumor lysis syndrome. This is a serious side effect that can cause kidney failure and heart and nervous system problems. You will be given plenty of fluids and medicines like allopurinol or rasburicase to prevent uric acid buildup.

Maintenance chemo

After the first, more intensive phases of treatment, lower-dose maintenance chemo is usually given for about 2 years to lower the risk of the disease coming back.

If the disease doesn’t go away or comes back

If the disease doesn’t respond to treatment or comes back, your options might include:

• The chemo drug nelarabine (Arranon), which is used to treat T-cell lymphoblastic disease
• Other chemo drugs

If the disease responds, high-dose chemo followed by a stem cell transplant may be an option. Clinical trials of newer treatments may also be a good option.