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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 non-Hodgkin lymphoma (NHL), accounting for about 1–2% of all lymphomas. It is a B-cell lymphoma. Lymphoplasmacytic lymphoma may also be called immunocytoma or Waldenstrom’s macroglobulinemia (WM).

With lymphoplasmacytic lymphoma, the lymphoma cells are usually found in the bone marrow, lymph nodes and spleen. This type of NHL usually occurs in older adults.

Large amounts of a protein called immunoglobulin M (IgM or macroglobulin) are made by the lymphoma cells. Increased amounts of this protein in the blood can cause the blood to thicken (hyperviscosity syndrome) and lead to problems with circulation, such as decreased blood flow to organs like the brain or eyes. This can cause:

  • dizziness
  • headache
  • blurring or loss of vision
  • numbness and tingling of the fingers or toes (peripheral neuropathy) and other neurologic symptoms

People with lymphoplasmacytic lymphoma tend to bleed easily. They also have fatigue and weakness.

Lymphoplasmacytic lymphoma is usually a slow-growing (indolent) lymphoma. Sometimes it can change (transform) into an aggressive large cell lymphoma.

Treatment

Lymphoplasmacytic lymphoma is treated like other slow-growing (indolent) lymphomas, with a few differences. There are several treatment options for lyphoplasmacytic lymphoma.

Watchful waiting

Like other indolent lymphomas, people with lymphoplasmacytic lymphoma who do not have any symptoms and whose cancer does not seem to be progressing often do not need treatment right away. However, treatment is given if there are symptoms, such as hyperviscosity syndrome, or when the cancer seems to be progressing.

Plasmapheresis

People with lymphoplasmacytic lymphoma who have signs or symptoms of hyperviscosity syndrome often have a procedure called plasmapheresis. Plasmapheresis can relieve their symptoms rapidly, before other treatments like chemotherapy have had a chance to work. Plasmapheresis temporarily lowers IgM levels by removing some of the abnormal IgM from the blood, which makes the blood thinner. However, plasmapheresis does not affect the lymphoma cells. Additional treatment, such as chemotherapy or biological therapy, is needed or the IgM protein will increase again. Plasmapheresis is usually given until chemotherapy starts to work.

During plasmapheresis, a machine is used to separate the plasmaplasmaThe liquid part of blood that carries the blood cells. Plasma contains many proteins and minerals. from the blood cells. The part of the blood that contains the abnormal protein is removed and blood cells are mixed with new plasma and given back to the person. A central venous catheter or 2 intravenous lines are needed to remove and return the blood to the body. Each plasmapheresis treatment takes a few hours.

Plasmapheresis is combined with chemotherapy to control the disease for a longer period of time.

Chemotherapy

People with lymphoplasmacytic lymphoma who have symptoms or hyperviscosity syndrome are usually started on chemotherapy. Drugs that may be used are:

  • chlorambucil (Leukeran) or cyclophosphamide (Cytoxan, Procytox), with or without prednisone (Deltasone)
  • fludarabine (Fludara) or cladribine (2-CDA, Leustatin)

These chemotherapy drugs may be used alone or in combination with other drugs. For example, CVP – cyclophosphamide, vincristine (Oncovin) and prednisone with rituximab (Rituxan) – is one combination that may be used to treat lymphoplasmacytic lymphoma.

Biological therapy

Monoclonal antibodies are a type of biological therapy that is effective in treating certain types of NHL. Rituximab (Rituxan) may be used alone or in combination with chemotherapy. It seems to improve the outcome for people with lymphoplasmacytic lymphoma.

Stem cell transplant

A stem cell transplant (SCT) is occasionally used to treat some people with lymphoplasmacytic lymphoma. It may be used for people whose lymphoma relapses or is not responding to other treatments (refractory). Many people with lymphoplasmacytic lymphoma are older or may not be in good health, so a SCT may not be a suitable option for them.

For more detailed information on specific drugs, go to sources of drug information.

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