Treating Lymphoma in Children

For children with classic Hodgkin lymphoma (cHL), combination chemo with or without immunotherapy or targeted therapy is often the main treatment. Radiation may be used, but doctors try to avoid giving it to children and adolescents when possible.

The outlook for HL with current treatments is quite good. A number of treatment combinations have been effective. Common initial regimens for children and adolescents with Hodgkin lymphoma include:

Early disease

• OEPA (vincristine, etoposide, prednisone, doxorubicin)
• OEPA/COPDAC (vincristine, etoposide, prednisone, doxorubicin, cyclophosphamide, dacarbazine)
• ABVE-PC (doxorubicin, bleomycin, vincristine, etoposide, prednisone, and cyclophosphamide)

Advanced disease

• OEPA/COPDAC (vincristine, etoposide, prednisone, doxorubicin, cyclophosphamide, dacarbazine)
• Bv-AVE-PC (brentuximab, doxorubicin, vincristine, etoposide, prednisone, cyclophosphamide)
• Bv-AVD (brentuximab, doxorubicin, vinblastine, dacarbazine)
• Nivo-AVD (nivolumab, doxorubicin, vinblastine, dacarbazine)
• AEPA/CAPDAC (brentuximab, etoposide, prednisone, doxorubicin, cyclophosphamide, dacarbazine)

These drug combinations may be used with or without radiation, depending on the extent of disease and how well it responds to chemo and other treatments.

NLPHL is very rare in children. In the early stages, surgery to remove the affected lymph node may be the only treatment needed. After surgery, the child is watched closely for signs of lymphoma. Chemo can be used if it comes back.

For early disease that cannot be removed with surgery alone and is not bulky, treatment might include:

• AVPC (doxorubicin, vincristine, prednisone, cyclophosphamide) with or without radiation
• CVbP (cyclophosphamide, vinblastine, prednisolone) with or without rituximab and/or radiation
• OEPA (vincristine, etoposide, prednisone, doxorubicin) with or without rituximab and/or radiation

There is little known about how to treat advanced NLPHL. It may be treated like classical Hodgkin lymphoma.

Stages I and II

In general, treatment for early stage LBL is similar to the treatment of acute lymphoblastic leukemia (ALL).

Chemotherapy is given in 3 phases: induction, consolidation, and maintenance. Many chemo drugs are used. The entire length of treatment is typically 2 to 3 years.

During this treatment, a child gets chemo in 2 ways:

• Systemically through their bloodstream or by mouth, and
• Intrathecally into their cerebrospinal fluid (the fluid that bathes the brain and spinal cord)

Shorter and less intensive treatments have also been used. For example, the chemo combination called CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) followed by maintenance therapy.

Stages III and IV

The treatment for children with advanced LBL is similar to the treatment given for stages I and II, although it might be more intensive. This is very similar to the treatment of high-risk acute lymphoblastic leukemia (ALL).

In some cases, radiation therapy may be given to the brain and spinal cord as well.

The main form of treatment for these lymphomas is chemotherapy, often along with the monoclonal antibody rituximab.

Stages I and II

Chemo is the main treatment of these lymphomas, but surgery might be done before chemo if the tumor is in only one area, such as a large abdominal (belly) tumor.

Several different chemo drugs are used.

For low-stage tumors that can be removed with surgery, treatment might include drugs such as cyclophosphamide, vincristine, prednisone, and doxorubicin, with or without intrathecal chemo (chemo given by spinal tap). This treatment lasts 6 to 9 weeks.

For low-stage tumors that can’t be removed with surgery or stage II tumors outside the belly, longer chemo treatment plans may be needed. These may include other chemo drugs like methotrexate and cytarabine, and an immunotherapy drug called rituximab.

Stages III and IV

Children with more advanced lymphomas are generally treated with rituximab plus more intensive chemotherapy. Because these lymphomas tend to grow quickly, the chemo cycles are short, with little rest between courses of treatment.

During this treatment, a child gets chemo in 2 ways:

• Systemically through their bloodstream or by mouth, and
• Intrathecally into their cerebrospinal fluid (the fluid that bathes the brain and spinal cord)

One treatment plan known as the French LMB protocol regimen alternates between different combinations of drugs every 3 to 4 weeks for a total of about 6 to 8 months.

These tumors are rare in children. There is no standard treatment for pediatric-type follicular lymphoma.

Stage I: Children with stage I disease who can have their tumors removed by surgery may be observed without additional treatment, unless there are signs the tumor has come back.

Higher stage: Higher-stage disease is often treated with chemotherapy, with or without immunotherapy (rituximab).

These tumors are rare in children and are typically early stage. There is no standard treatment for pediatric marginal zone lymphomas.

• Many of these nodal tumors can be cured with surgery or radiation alone.
• Other options include chemo and immunotherapy (rituximab).

A special type of marginal zone lymphoma in the stomach, called MALT, is also treated with antibiotics.

Chemotherapy (chemo) and targeted therapies are the main treatments for ALCL.

Treatment for these lymphomas usually involves combinations of chemo with or without targeted treatments. Targeted therapies that have been effective for ALCL treatment include brentuximab vedotin and ALK inhibitors (such as crizotinib).

There is no standard strategy for ALCL treatment in children, but the following combinations have been used successfully:

• APO (doxorubicin, prednisone, vincristine)
• ALCL99 (dexamethasone, cyclophosphamide, ifosfamide, etoposide, doxorubicin, methotrexate, cytarabine, prednisolone, and vinblastine)

Depending on the treatment plan, therapy can last from 4 months to 1 year.

There is no standard treatment for peripheral T-cell lymphoma. Doctors have had some success treating it with chemo regimens similar to those used for acute lymphoblastic leukemia and non-Hodgkin lymphomas.

Radiation or stem cell transplant may also be treatment options.

There is no standard treatment for cutaneous T-cell lymphoma. In some cases, observation may be recommended.

Treatment is often needed if a child develops hemophagocytic syndrome, a complication of the disease in which the immune system begins to attack the body. Medications to calm the immune system response, called immunomodulatory drugs, might be used. Chemo might also be an option.

Not much is known about CNS lymphoma in children.

Treatment options include chemo drugs that can reach the brain, such as high doses of cytarabine or methotrexate, or intrathecal chemo (given by a spinal tap) when cancer cells are found in the cerebrospinal fluid (CSF).

Stem cell transplant, whole-brain radiation, or clinical trials may also be treatment options.

There is no standard treatment for pediatric gray zone lymphoma.

Because it has features of B-cell lymphoma and Hodgkin lymphoma, treatment is often similar to the treatments used for these cancers.