Symptoms of Waldenström’s macroglobulinemia usually begin gradually. Common symptoms are fatigue and loss of energy due to anemia. Bleeding from the nose and gums may also occur, and tingling in the fingers and toes is often seen (peripheral neuropathy). Examination may also reveal enlarged liver, spleen, or lymph nodes. Blurring or vision impairment may also occur due to changes in blood flow through the blood vessels that serve the retina. The symptoms are quite variable depending on the effect of the thickened (viscous) blood on the organ involved.
The causes of Waldenström’s macroglobulinemia are unknown. There is evidence of occurrence within families, but such occurrence is uncommon.
Waldenström’s macroglobulinemia is a very rare disorder affecting about 1 in 3.4 million American men and about half that number of American women. The incidence of WMG is estimated to be about 5 per 1,000,000 people over the age of 50. The median age at diagnosis is 67. However, cases have been diagnosed in patients in their twenties. This disorder is considerably less common among people of African descent.
When patients show symptoms of an enlarged spleen and liver combined with bleeding of the retina, WMG is reasonably suspected. The results of a complete blood count (CBC) usually show low red blood cell counts as well as low platelet counts. In such circumstances, electrophoresis (subjecting blood plasma to an electric impulse) of serum samples will show a peak reading for IgM.
With asymptomatic patients, a period of watchful waiting is considered the preferred course. To reduce the blood thickness, plasmapheresis, a method of blood exchange which filters out much of the IgM protein, has been shown to be effective in preventing complications of hyperviscosity. The blood cells are separated from the liquid component (serum or plasma). The heavy IgM molecules that cause the trouble are discarded with the liquid fraction of the exchange. The blood is “reconstituted” by adding previously donated plasma or a plasma substitute. If the disorder progresses, red blood cell transfusions and/or platelet infusions may be necessary.
Traditional chemotherapeutic agents, particularly chlorambucil (Leukeran), cyclophosphamide (Cytoxan), and/or melphalan (Alkeran) either as single agents or in combination with others, have been used in the management of this disease for over 40 years. The monoclonal antibody, rituximab, has been found to be effective. Ongoing research is examining combinations of these drugs. Many new therapies have been introduced in the past few years and include fludarabine, cladribine, bortezomib, carfilzomib, lenalidomide, bendamustine and everolimus. FDA has approved the use of ibrutinib for Waldenstrom Macroglobulinemia.
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Peripheral blood stem transplant and bone marrow transplant, using the patient’s own stem cells or marrow and that of related or unrelated donors, has been used but the procedure remains highly controversial.
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