Aggressive non-Hodgkin lymphoma (NHL) progresses rapidly. It makes up about 60 percent of all NHL cases in the United States. Aggressive subtypes include:
- AIDS-associated lymphoma
- Burkitt lymphoma
- Central nervous system (CNS) lymphoma
- Diffuse large B-cell lymphoma (DLBCL)
- Mantle cell lymphoma
- Peripheral T-cell lymphoma (PTCL)
Patients with fast-growing NHL are frequently treated with chemotherapy that consists of four or more drugs. In most cases, this is the combination therapy called R-CHOP (rituximab [Rituxan®], cyclophosphamide [Cytoxan®], doxorubicin [hydroxydoxorubicin], Oncovin® [vincristine] and prednisone). This intensive, multidrug chemotherapy can be very effective for aggressive lymphoma, and cures have been achieved. Chemotherapy can be supplemented by radiation therapy in select cases, for instance, when large NHL masses are found during the diagnostic and staging process.
Treating Specific Aggressive Subtypes
The types of NHL that are most often seen in people with acquired immune deficiency syndrome (AIDS) are diffuse large B-cell lymphoma, Burkitt lymphoma and primary central nervous system (CNS) lymphoma. Treatment outcomes depend on how well the patient with AIDS responds to therapy and manages the effects of chemotherapy on blood counts. Because AIDS already leads to low blood cell counts, chemotherapy must be carefully considered to determine whether the chemotherapy's additional effects on blood levels can be managed. The number of people developing AIDS-associated NHL has decreased in the last several years because of improved treatment of HIV, the virus that can lead to AIDS.
This B-cell subtype is considered to be highly aggressive. It may involve the jaw, bones of the face, bowel, kidneys, ovaries, bone marrow, blood, central nervous system (CNS) and other organs. Burkitt lymphoma was named after Dr. Dennis Burkitt, a surgeon who first identified the disease in children in Africa. In Africa, the disease usually appears in children as a mass in a facial bone, especially the jaw.
Doctors typically use highly aggressive chemotherapy to treat this subtype of NHL. Commonly used regimens include:
- CODOX-M/IVAC (cyclophosphamide, vincristine, doxorubicin and high-dose methotrexate) alternating with IVAC (ifosfamide, etoposide and high-dose cytarabine)
- Hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin and dexamethasone) alternating with methotrexate and cytarabine
- DA-EPOCH-R (dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin plus rituximab)
Participation in clinical trials is recommended for all patients with Burkitt lymphoma.
There are two types of central nervous system (CNS) lymphoma: primary and secondary. Primary CNS lymphoma starts in the brain and/or the spinal cord. It is often a feature of AIDS-associated lymphoma, although it may be related to other NHL subtypes as well. Secondary CNS lymphoma starts in another area of the body and spreads to the brain and/or spinal cord.
Both primary and secondary CNS lymphomas are uncommon. Standard treatment may include chemotherapy, glucocorticoid drugs and/or radiation therapy. Immunotherapy and high-dose chemotherapy with stem cell transplantation are being studied in clinical trials. Read more>>
Diffuse large B-cell lymphoma (DLBCL) is the most common NHL subtype, making up about 30 percent of all NHL cases in the United States. It affects mostly middle-aged and older adults. DLBCL usually starts in lymph nodes in the neck or abdomen and is characterized by masses of large B cells (lymphocytes). DLBCL grows rapidly and frequently involves the spleen, liver, bone marrow or other organs.
For some patients, DLBCL may be the initial diagnosis. For other patients, their indolent lymphomas such as small lymphocytic lymphoma or follicular lymphoma transform and become DLBCL.
DLBCL is frequently treated with a chemotherapy regimen that is made up of four or more drugs. One regimen is R-CHOP. Another more intense regimen is DA-EPOCH-R, which is a combination of dose-adjusted etoposide (Etopophos®, VePesid®, Toposar®), prednisone, vincristine (Oncovin®), cyclophosphamide, hydroxydoxorubicin (doxorubicin) plus rituximab.
Mantle cell lymphoma begins in the lymph nodes and spreads to the spleen, blood, bone marrow and sometimes the esophagus, stomach and intestines. This subtype originates from a lymphocyte in the mantle zone of the lymph node. This subtype usually occurs in people over age 50 and is found in more frequently in men than in women. Standard treatment is combination chemotherapy, either with or without autologous stem cell transplantation.
To read more about mantle cell lymphoma and treatment options, download or order The Leukemia & Lymphoma Society's (LLS's) free fact sheet Mantle Cell Lymphoma Facts.
Peripheral T-cell lymphoma (PTCL) refers to a group of aggressive NHL subtypes that originate in mature T-cell lymphocytes. PTCL generally affects people age 60 and older. The most common subtypes include:
- Peripheral T-cell, not otherwise specified (PTCL NOS) - This is the most common subtype of PTCL. It often involves lymph node sites but can also involve other areas such as the liver, bone marrow, gastrointestinal track and skin.
- Anaplastic Large Cell Lymphoma (ALCL) - This subtype usually starts in the lymph nodes and can spread to the skin. There are two main subtypes of ALCL:
- Systemic ALCL ALK-1 positive anaplastic large cell lymphoma - About 80 percent of patients with this subtype are cured. This disease is more common in young people.
- Systemic ALCL ALK-1 negative anaplastic large cell lymphoma - This subtype occurs mainly in older patients. Treatment with chemotherapy or radiation therapy is often less successful and a stem cell transplant may be discussed.
- Primary cutaneous anaplastic large cell lymphoma - This subtype mostly affects the skin, but other parts of the body may be involved.
- Hepatosplenic T-cell lymphoma - This subtype of PTCL starts in the liver and spleen and usually affects young men.
- Angioimmunoblastic T-cell lymphoma - This type of T-cell lymphoma often involves lymph nodes and the bone marrow. Many patients have “paraneoplastic symptoms” including fevers, rashes and abnormal protein levels in their blood.
- Extranodal natural killer/T-cell lymphoma - This subtype of lymphoma can occur in the nasal sinuses or in other parts of the body. It is usually a very aggressive lymphoma that requires both chemotherapy and radiation.
- Enteropathy-type intestinal T-cell lymphoma - This subtype develops in the small bowel of patients with untreated celiac disease.
PTCL is commonly treated with the regimens used for diffuse large B-cell lymphoma. Chemotherapy with CHOP is the standard treatment for newly diagnosed PTCL. However, clinical trials are under way to develop more effective treatment approaches, and patients are encouraged to seek out these clinical trials.
For more information about PTCL and treatment options, download or order LLS's free fact sheet Peripheral T-Cell Lymphoma Facts.