Lymphoplasmacytic Lymphoma (LPL) is an indolent (slow growing) cancer that affects plasma cells. A plasma cell is a specialized type of B cell that produces antibodies used for fighting infections. If a mutation occurs while the B cells are maturing into plasma cells, then they multiply uncontrollably.
As a result, they overproduce a protein called macroglobulin (IgM), which is a type of antibody. High IgM levels cause hyperviscosity of the blood, meaning that it becomes too thick. The thickness of the blood causes most of the symptoms a patient suffers from. Because of macroglobulin’s activity in this cancer, it is also called Waldenstrom’s Macroglobulinemia.
This rare disease represents only 1.5% of all Non-Hodgkin’s Lymphomas, and it is primarily diagnosed in older patients. Men are more likely to be diagnosed than women. Patients often have tumors in their lymph nodes, bone marrow, and spleen.
Patients with LPL do not always present with symptoms. However, some patients may experience one or all of the following:
Diagnosis of LPL is done using blood work and a bone marrow biopsy. Doctors will measure the levels of IgM and other tumor markers in the blood. Cells from the bone marrow biopsy will be examined under a microscope to confirm the LPL diagnosis.
Other tests such as CT scans, PET scans, chest X-rays, and ultrasounds may be used to rule out other similar cancers, such as Multiple Myeloma, and to stage the disease.
There are several treatments used for Waldenstrom's Macroglobulinemia. Your doctor may use one of the following, or multiple in combination:
Watch and Wait: This treatment option is used when patients do not show symptoms. They are monitored to make sure the lymphoma does not progress, but no further treatment is pursued unless tumors spread.
Chemotherapy: High-dose chemicals are injected into the body. These medicines attack fast growing cells, like tumors.
Stem Cell Transplantation: In some cases, chemotherapy will wipe out the immune system. In these cases, stem cell transplantation is required to return the patient to health.
Thalidomide and Bortezomib: These drugs are used for both LPL and Multiple Myeloma.
Splenectomy: The removal of the spleen is sometimes necessary.
Zevalin: The Zevalin radioimmunotherapy regimen is being researched as a treatment for lymphoplasmacytic lymphoma.
Rituxan: Rituxan helps your body’s immune system target cancerous cells and remove them.
Plasmapheresis: This treatment removes unhealthy cells and proteins from the blood. While this won’t remove the cancerous cells, it can filter out IgM, thus returning the blood to a normal thickness and alleviating some symptoms.
The ICD 10 Codie is C88. 0 which can be used to indicate a diagnosis for reimbursement purposes.
Sources: NCI Cancer Facts paper on Waldenström’s Macroglobulinemia, Lymphoplasmacytic Lymphoma at Wikipedia
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