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 can occur in both children and adults, and it can be considered either a lymphoma 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’s treated with intensive chemotherapy (chemo), when possible.

Combinations of many drugs are used. These can include cyclophosphamide, doxorubicin (Adriamycin), vincristine, L-asparaginase, methotrexate, prednisone, 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.

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 once it has spread to the bone marrow.

Peripheral T-cell lymphomas

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

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

Adult T-cell leukemia/lymphoma

This lymphoma is linked to infection with the HTLV-1 virus. There are 4 subtypes, and treatment depends on which subtype you have.

• The smoldering and chronic subtypes grow slowly. Like other slow-growing lymphomas (such as follicular lymphoma and small lymphocytic lymphoma), these subtypes are often watched without treatment 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. Another option is chemo, using the CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisone) or other combinations.
• The acute subtype also can be treated with either anti-viral drugs or chemo (typically the CHOP regimen). 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. It can also involve 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.

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 might be treated first with steroids (such as prednisone or dexamethasone) alone, especially in older patients who might have trouble tolerating chemo. This treatment can reduce fever and weight loss, but the effect is often temporary. If chemo is needed, combinations such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) may be used. Another option might be the chemo combination of cyclophosphamide, doxorubicin, and prednisone, along with the monoclonal antibody brentuximab vedotin (Adcetris). If the lymphoma is only in one area, radiation therapy may be an option.

Standard doses of chemo rarely produce long-term remissions, 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. Patients with stage I or II disease who aren't healthy enough for chemotherapy may be treated with radiation therapy alone. Most other patients are treated with chemoradiation (chemo and radiation given together) or chemo followed by radiation. Several different chemo combinations can be used.

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

Enteropathy-associated T-cell lymphoma

This lymphoma generally develops in the small intestine or colon. Intensive chemo using several drugs is usually the main treatment. Often CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) is the chemo used. If the lymphoma is only in one area, radiation therapy may be used as well. But if these treatments work, a hole (perforation) can 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 and is treated with chemo regimens such as CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) or CHOEP (cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisone). Another option might be the chemo combination of cyclophosphamide, doxorubicin, and prednisone, along with the monoclonal antibody brentuximab vedotin (Adcetris). Doctors might recommend radiation therapy as well for some patients.

This lymphoma often responds well to treatment, and long-term survival is common, especially if the lymphoma cells have too much of the ALK protein. If the cells lack the ALK protein or if the lymphoma returns after initial treatment, a stem cell transplant may be an option. Another option for lymphomas that no longer respond to initial treatment is brentuximab vedotin (Adcetris).

Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL): For ALCL that develops in the capsule (normal protective scar tissue) that forms around a breast implant, experts typically recommend removing the implant and the capsule surrounding it. Additional treatment might include chemo, sometimes with radiation.

Peripheral T-cell lymphoma, unspecified

These lymphomas are generally treated the same way as diffuse large B-cell lymphoma (DLBCL). Chemo with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or other drug combinations is used. For early-stage disease, radiation therapy may be added. Another option for some of these lymphomas might be the chemo combination of cyclophosphamide, doxorubicin, and prednisone, along with the monoclonal antibody brentuximab vedotin (Adcetris). A stem cell transplant may be recommended when possible.

If other treatments are no longer working, newer chemo drugs such as pralatrexate (Folotyn), targeted drugs such as bortezomib (Velcade) or belinostat (Beleodaq), or immunotherapy drugs such as alemtuzumab (Campath) and denileukin diftitox (Ontak) may be tried.

The outlook for these lymphomas is usually not as good as in DLBCL, so taking part in a clinical trial of newer treatments is often a good option.