Treating T-Cell Non-Hodgkin Lymphoma

Non-Hodgkin lymphoma (NHL) is generally divided into main 2 types, based on whether it starts in B lymphocytes (B cells) or T lymphocytes (T cells).

There are many different types of T-cell lymphomas, and treatment can vary based on which type you have.

T-lymphoblastic lymphoma/leukemia

This disease (also known as T-cell acute lymphoblastic leukemia/lymphoma) can be considered either a lymphoblastic lymphoma (LBL) or a type of acute lymphoblastic leukemia (ALL), depending on how much of the bone marrow is involved. (Leukemias have more bone marrow involvement.)

Regardless of whether it’s labeled as a lymphoma or a leukemia, this is a fast-growing disease that needs to be treated with intensive chemotherapy (chemo), when possible.

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). 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. Some doctors suggest maintenance chemo for up to 2 years after the initial treatment to reduce the risk of recurrence. High-dose chemo followed by a stem cell transplant may be another option.

Treatment is typically given in the hospital at first. During this time, patients are at risk for tumor lysis syndrome (described in Chemotherapy for Non-Hodgkin Lymphoma), so they are given plenty of fluids and drugs like allopurinol to prevent uric acid build up.

For more details on treatment, see Treating Acute Lymphocytic Leukemia (Adults) and Treating Childhood Leukemia.

Although this lymphoma is fast-growing, if it hasn’t spread to the bone marrow when it’s first diagnosed, the chance of cure with chemo is quite good. But it is harder to cure if it has spread to the bone marrow.

Peripheral T-cell lymphomas (PTCLs)

Cutaneous T-cell lymphomas (mycosis fungoides, Sezary syndrome, and other skin lymphomas)

Treatment of these skin lymphomas is discussed in Treating Lymphoma of the Skin.

Adult T-cell leukemia/lymphoma

Adult T-cell leukemia/lymphoma (ATLL) is linked to infection with the HTLV-1 virus. Treatment depends on which subtype of ATLL you have.

The smoldering and chronic subtypes grow slowly. Like other slow-growing lymphomas (such as follicular lymphoma and small lymphocytic lymphoma), these subtypes can often be watched without treating them right away, as long as they aren’t causing problems other than mildly swollen lymph nodes. If treatment is needed, one option is interferon and the anti-viral 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 (see below) or other combinations.

The acute subtype also can be treated with either anti-viral drugs or chemo (see below). If it responds well to treatment, a stem cell transplant might be considered.

Anti-viral therapy is not helpful for the lymphoma subtype, so it is typically treated with chemotherapy (see below). It can also invade the tissues around the brain and spinal cord, so chemo is given into the spinal fluid (intrathecal chemo) as well. Treatment after chemo may include a stem cell transplant.

Common chemo regimens for ATLL (regardless of the subtype) 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
• HyperCVAD (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.

Because there is no clear standard treatment for this disease, patients might want to consider enrolling in a clinical trial, if one is available.

Angioimmunoblastic T-cell lymphoma

This fast-growing lymphoma is typically treated with intense chemo, using a combination of drugs such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or CHOEP (CHOP plus etoposide). Another option might be the combination of the chemo drugs cyclophosphamide, doxorubicin, and prednisone (CHP), along with the monoclonal antibody brentuximab vedotin (Adcetris), if the lymphoma cells have the CD30 protein.

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.

Standard doses of chemo might not lead to a long-term remission, so a stem cell transplant is often suggested after initial chemotherapy if a person can tolerate it.

Extranodal natural killer/T-cell lymphoma, nasal type

This rare lymphoma is often confined to the nasal passages.

People with stage I or II disease who aren't healthy enough for chemotherapy may be treated with radiation therapy alone. Most other people are treated with chemotherapy (chemo), followed by radiation therapy (chemoradiation). Several different combinations of chemo drugs can be used.

For more advanced (stage IV) lymphomas, chemotherapy, with or without radiation, is typically the main treatment. Again, several combinations of chemo drugs can be used.

If the lymphoma doesn’t go away completely, or if it was initially stage IV, a stem cell transplant may be done if possible.

Enteropathy-associated T-cell lymphoma

This lymphoma generally develops in the small intestine or colon in people with celiac disease.

Intensive chemo using several drugs is usually the main treatment. Commonly used combinations include CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or CHOEP (CHOP plus etoposide). Another option might be the combination of the chemo drugs cyclophosphamide, doxorubicin, and prednisone (CHP), along with the monoclonal antibody brentuximab vedotin (Adcetris), if the lymphoma cells have the CD30 protein.

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 concern if these treatments work is that a hole (perforation) might develop in the intestines (as the lymphoma cells die), so surgery might be done first to remove the part of the intestines containing the lymphoma. Surgery may also be needed before chemo or radiation if a person is diagnosed with this lymphoma because it caused a perforation or intestinal blockage (obstruction).

A stem cell transplant may be an option if the lymphoma responds to chemo.

Anaplastic large cell lymphoma (ALCL)

This fast-growing lymphoma mainly affects lymph nodes.

Treatment typically includes intense chemotherapy (chemo) using a combination of several drugs, sometimes along with radiation therapy if the lymphoma is confined to one or few areas.

Common chemo regimens for ALCL include CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) or CHOEP (CHOP plus etoposide). Another option might be CHP (cyclophosphamide, doxorubicin, and prednisone) along with the monoclonal antibody brentuximab vedotin (Adcetris).

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

If the lymphoma returns after initial treatment, other chemo drugs (or other types of drugs) might be options, either alone or in combination. The options will depend to some extent on if the plan is for the person to eventually get a stem cell transplant.

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

For ALCL that develops in the capsule (scar tissue) that forms around a breast implant, doctors typically remove the implant and the capsule surrounding it. If not all of the lymphoma can be removed with surgery, radiation therapy might be given to the area. Additional treatment might include chemo, typically with one of the same regimens listed above for ALCL (CHOP, CHOEP, CHP plus brentuximab vedotin, or brentuximab vedotin alone), and sometimes with radiation.

Peripheral T-cell lymphoma, not otherwise specified

This type of lymphoma is typically treated with intense chemotherapy (chemo), using a combination of drugs such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or CHOEP (CHOP plus etoposide). Another option might be the combination of the chemo drugs cyclophosphamide, doxorubicin, and prednisone (CHP), along with the monoclonal antibody brentuximab vedotin (Adcetris), if the lymphoma cells have the CD30 protein. For early-stage disease, radiation therapy may be given to the site of the lymphoma as well.

For people who can’t tolerate intense chemo, a single chemo, immunotherapy, or targeted drug might be an option. If the lymphoma is only in one area, radiation therapy may be an option.

If the lymphoma responds to treatment, a stem cell transplant may be recommended when possible.

If treatments above are no longer working, other chemo, immunotherapy, or targeted drugs, either alone or in combination, can often be tried. The options will depend to some extent on if the plan is for the person to eventually get a stem cell transplant.

Because these lymphomas can often be hard to treat, taking part in a clinical trial of newer treatments is often a good option.