Treating T-Cell Non-Hodgkin Lymphomas

Precursor T-lymphoblastic lymphoma/leukemia

This disease can occur in both children and adults and it can be considered either a lymphoma or leukemia.

It’s considered a lymphoma if there are tumor masses and if cancer (lymphoma) cells make up less than 25% of the bone marrow. This is a fast-growing disease that’s treated with intensive chemo when possible.

The treatment for the lymphoma form of this disease is similar to that used for the leukemia form, which is discussed in more detail in Acute Lymphocytic Leukemia in Adults and Childhood Leukemia.

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.

During the initial treatment, patients are at risk for tumor lysis syndrome (described in the Chemotherapy for Non-Hodgkin Lymphoma section), and so are given plenty of fluids and drugs like allopurinol.

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 once it has spread to the bone marrow, only about 40% to 50% of patients can be cured.

Peripheral T-cell lymphomas

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

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

Adult T-cell leukemia/lymphoma

There are 4 subtypes of this lymphoma, 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 watched without treatment as long as they aren’t causing problems other than mildly swollen lymph nodes. If treatment is needed, one option is treatment with interferon and zidovudine to fight the HTLV-1 virus. If the lymphoma is affecting the skin, it may be treated with radiation. Another option is chemo, using CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or other combinations.

The acute and lymphoma subtypes also can be treated with either antiviral drugs or chemo. Some doctors feel that the acute subtype responds better to antiviral drugs, CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) is the most common chemo combination used.

The lymphoma subtype can involve the tissues around the brain and spinal cord, so chemo is also given into the spinal fluid (intrathecal chemo). Treatment after chemo may include an allogeneic stem cell transplant. Because there is no clear standard treatment for this disease, patients should consider enrolling in a clinical trial if one is available.

Angioimmunoblastic T-cell lymphoma

This fast-growing lymphoma is often first treated with steroids (such as prednisone or dexamethasone) alone, especially in older patients who would 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. If the lymphoma is only in one area, radiation therapy may be an option.

Chemo rarely produces 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 disease who do not have any poor risk factors, such as being 60 years or older in age, having B symptoms, or certain lab values, such as a high LDH (lactate dehydrogenase) or Epstein-Barr virus level, may be treated with radiation therapy alone. Patients with more advanced stage disease or stage I with poor risk factors can be treated with chemoradiation (chemo and radiation given together), chemo followed by radiation, or chemo alone.

When chemoradiation is given, it may be cisplatin with radiation followed by chemo with VIPD (etoposide, ifosfamide, carboplatin and dexamethasone). Another option is radiation with DeVIC (dexamethasone, etoposide, ifosfamide, and carboplatin).

If chemo is given before radiation, often DeVIC or SMILE (methotrexate, dexamethasone, ifosfamide, leucovorin, etoposide, and L-asparaginase).

SMILE or AspaMetDex (L-asparaginase, methotrexate, and dexamethasone) are options if chemo is given without radiation.

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. 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 often surgery to remove the part of the intestines containing the lymphoma is done first. Surgery may also be needed before chemo or radiation if the patient 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

This fast-growing lymphoma mainly affects lymph nodes and is treated with CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone), CHOEP (cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisone), or some other chemo regimens. 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 stain positive for the ALK protein. If the cells lack the ALK protein or if the lymphoma returns after initial treatment, an autologous stem cell transplant may be an option. Another option for lymphomas that no longer respond to initial treatment is the labeled monoclonal antibody brentuximab vedotin (Adcetris).

For anaplastic large cell lymphoma associated with a breast implant, experts recommend removing the implant and the capsule surrounding it (that contains the lymphoma). Additional treatment may include chemo, sometimes with radiation.

Peripheral T-cell lymphoma, unspecified

These lymphomas are generally treated the same way as diffuse large B-cell lymphomas. Chemo with CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or other drug combinations is used. For early-stage disease, radiation therapy may be added. Stem cell transplants may be recommended as part of the treatment when possible.

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

The outlook is usually not as good as in diffuse B-cell lymphoma, so taking part in a clinical trial of newer treatments is often a good option.

The American Cancer Society medical and editorial content team
Our team is made up of doctors and master’s-prepared nurses with deep knowledge of cancer care as well as journalists, editors, and translators with extensive experience in medical writing.

Last Medical Review: August 26, 2014 Last Revised: February 29, 2016

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