Non-Hodgkin lymphoma

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Lymphoplasmacytic lymphoma

Lymphoplasmacytic lymphoma gets its name because the lymphoma cells have characteristics of both lymphocytes and plasma cells. It is an uncommon type of B-cell non-Hodgkin lymphoma (NHL).

Lymphoplasmacytic lymphoma usually occurs in older adults. The average age at diagnosis is 60. It is usually slow growing (indolent). But it can sometimes change into a fast-growing (aggressive) type of NHL.

Symptoms

With lymphoplasmacytic lymphoma, the lymphoma cells are usually found in the bone marrow, lymph nodes and spleen. People with lymphoplasmacytic lymphoma tend to bleed easily. They also have fatigue and weakness. Most people with lymphoplasmacytic lymphoma do not have B symptoms (unexplained fever, drenching night sweats and unexplained weight loss).

The lymphoma cells make varying amounts of a protein called immunoglobulin M (IgM, or macroglobulin). Higher than normal amounts of this protein in the blood can make it thick (called hyperviscosity syndrome). When this occurs, the condition is called Waldenstrom macroglobulinemia. This can lead to problems with circulation and less blood flow to the brain, the eyes or other organs, which can cause:

  • dizziness
  • headache
  • blurred vision or loss of vision
  • shortness of breath
  • numbness and tingling of the fingers or toes (called peripheral neuropathy)
  • muscle weakness
  • confusion

Treatments

You may be offered one or more of the following treatments for lymphoplasmacytic lymphoma.

Watchful waiting

Watchful waiting (also called active surveillance) may be offered for lymphoplasmacytic lymphoma because it develops slowly and may not need to be treated right away. The healthcare team will carefully monitor the person with lymphoplasmacytic lymphoma and start treatment when symptoms appear, such as hyperviscosity syndrome, or there are signs that the disease is progressing more quickly.

Chemotherapy

People with lymphoplasmacytic lymphoma who have symptoms or hyperviscosity syndrome are usually given chemotherapy. Chemotherapy drugs that may be used with or without prednisone include:

  • chlorambucil (Leukeran)
  • fludarabine (Fludara)
  • bendamustine (Treanda)
  • cyclophosphamide (Cytoxan, Procytox)

Combinations of chemotherapy drugs that may be used include:

  • DRC – dexamethasone (Decadron, Dexasone), rituximab (Rituxan) and cyclophosphamide
  • BRD – bortezomib (Velcade) and rituximab, with or without dexamethasone
  • CVP – cyclophosphamide, vincristine (Oncovin) and prednisone
  • R-CVP – CVP with rituximab
  • thalidomide (Thalomid) and rituximab

Targeted therapy

Targeted therapy uses drugs to target specific molecules (such as proteins) on the surface of cancer cells. These molecules help send signals that tell cells to grow or divide. By targeting these molecules, the drugs stop the growth and spread of cancer cells while limiting harm to normal cells.

Targeted therapy drugs used alone or in combination to treat lymphoplasmacytic lymphoma include rituximab, bortezomib and ibrutinib (Imbruvica).

Immunotherapy

Immunotherapy works by stimulating, boosting, restoring or acting like the body’s immune system to create a response against cancer cells. Immunomodulatory drugs are a type of immunotherapy that interferes with the growth and division of cancer cells.

Thalidomide is a type of immunomodulatory drug that may be used to treat lymphoplasmacytic lymphoma.

Radiation therapy

External beam radiation therapy may be used to treat lymphoplasmacytic lymphoma that develops outside of the lymphatic system (called extralymphatic disease), but this is rare.

Stem cell transplant

Some people with lymphoplasmacytic lymphoma may be offered a stem cell transplant. It may be used if the lymphoma comes back (recurs) after treatment or doesn’t respond to other treatments (called refractory disease). Many people with lymphoplasmacytic lymphoma are older or may not be in good health, so a stem cell transplant may not be a good treatment option for them.

Stories

Dr Shawn Li I’m extremely grateful to the Canadian Cancer Society for funding my research with an Innovation Grant.

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