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Waldenström’s Macroglobulinaemia (lymphoplasmacytic lymphoma)

Waldenström’s Macroglobulinaemia, is a rare, slow growing form of non-Hodgkin lymphoma. 

It is a rare, slow growing tumour of cells involved in the immune system. These cells are known as B-cells (or B-lymphocytes) and normally make antibodies. It is classified by the World Health Organisation (WHO) as a subtype of non-Hodgkin lymphoma. 

How does Waldenström's Macroglobulinaemia affect the body?

Waldenstrom's Macroglobulinaemia (WM) features an abnormal proliferation of lymphoplasmacytic cells. These cells produce an abnormal antibody called IgM.

If large amounts of abnormal IgM are produced, the blood can becomes too 'sticky' (hyperviscosity) making blood flow to the body’s organs difficult. This complication occurs in approximately 10-30% of people with this disease. Symptoms caused by hyperviscosity may include changes in vision, headaches, hearing loss, fatigue; abnormal bleeding and confusion. There is also an increased risk of infection.

Who does Waldenström's Macroglobulinaemia commonly affect?

WM is rare, accounting for approximately 1-2% of all blood cancers. The average age at diagnosis is 63. It is slightly more common in men. 

Do we know what causes Waldenström's Macroglobulinaemia?

Whilst the exact cause is not known, WM is not due to infection and cannot be passed from one person to another.

How is Waldenström's Macroglobulinaemia treated?

There is currently no known cure for WM. Several treatment options exist to prevent or control symptoms of the disease, and to destroy the tumour cells that make the abnormal IgM protein. WM often develops slowly, so treatment may not be needed straight away. People who are without symptoms are generally managed by the 'watch and wait' (also called Active Monitoring) approach.

Once symptoms develop or the disease progresses, chemotherapy is used to treat the disease. This may include FCR (fludarabine, cyclophosphamide and rituximab). Treatment that stimulates the immune system to fight cancer, biological therapy, is also being used to treat WM. Promising results have been seen when chemotherapy and biological therapy are used in combination.

People presenting with high levels of IgM and hyperviscosity syndrome (thickening of the blood) are likely to undergo plasmapheresis. This is where blood is circulated through a machine which removes the thick IgM protein-containing plasma. It is then replaced with a plasma substitute

Other treatment for WM may include surgical removal of the spleen (splenectomy), red blood cell transfusions, radiation therapy, clinical trials, and stem cell transplantation.