Lymphoplasmacytic lymphoma is one type of non-Hodgkin lymphoma (NHL).
From a practical viewpoint doctors divide all the various types of NHL into one of three groups: low grade, high grade or intermediate grade. This division is based on the appearances under the microscope and helps predict how the condition will behave.
Low grade NHLs usually have a relatively slow growth rate, running a long course and often needing little or no treatment for months or possibly years. When they do need treatment they respond well but are likely to come back again at some time in the future. They frequently respond to treatment on several occasions over the years but may eventually become resistant or transform to a high grade form of NHL.
High grade types of NHL usually grow more rapidly and behave more aggressively and so need immediate treatment. Despite this they are more likely to be completely cured than low grade NHLs. Around half of all people with high grade forms of NHL will be cured by the use of intensive chemotherapy.
Intermediate types of NHL lie somewhere between these two extremes in their behaviour.
Lymphoplasmacytic lymphoma is a low grade lymphoma and usually only progresses very slowly. Because of this treatment is often delayed until the condition causes symptoms.
When treatment is needed chemotherapy is used. Lymphoplasmacytic lymphoma is very sensitive to chemotherapy and often disappears with simple drug treatment. This is frequently given in tablet form, using a drug called chlorambucil, or alternatively injections of either fludarabine or cladribine may be given.
Occasionally, in younger patients, bone marrow transplantation may be considered as a treatment option.
If a hyperviscosity syndrome develops this may be treated with chemotherapy but sometimes removal of the abnormal protein from the blood is also needed. This is done by a plasmapheresis. This involves placing two needles into veins in the arm, these are then connected to a machine that takes the blood from one vein, washes out the abnormal protein and returns it to the other vein. The benefits of plasmapheresis are almost immediate.
Although treatment normally leads to a rapid remission lymphoplasmacytic lymphoma almost always comes back again, although it may be years before it reappears. Further chemotherapy can then be given and another remission obtained.
This pattern repeats itself over many years. This means that although it is still doubtful whether it is ever possible to permanently cure lymphoplasmacytic lymphoma the great majority of patients can have their disease controlled, and lead a relatively normal life, for many years.