Waldenstrom macroglobulinemia

Waldenström macroglobulinemia (WM) is a hematological malignancy involving lymphocytes. It is a type of nonaggressive non-Hodgkin lymphoma. It is also classified as a lymphoplasmacytic lymphoma. more...

It is named after the Swedish physician Jan G. Waldenström (1906-1996), who identified the condition.

Incidence/Prevalence

WM is a rare disorder, with 1,400 cases occurring in the United States annually.

Symptoms

Symptoms of WM include weakness, fatigue, weight loss and chronic oozing of blood from the nose and gums. Peripheral neuropathy can occur in 10% of patients. Some of these symptoms are due to the effects of the IgM paraprotein, which may cause autoimmune phenomenon or cryoglobulinemia.

Unique to WM is the occurrence of the hyperviscosity syndrome. This is attributed to the IgM monoclonal protein increasing the viscosity of the blood. Symptoms of this are mainly neurologic and can include blurring or loss of vision, headache. Rarely this can lead to stroke or coma.

Diagnosis

A distinguishing feature of WM is the presence of an IgM monoclonal protein (or paraprotein) that is produced by the cancer cells.

Prognosis

Median survival is approximately 5 years from time of diagnosis. New treatments have made longer term survival a reality for many with this condition.

Treatment

Treatment includes the monoclonal antibody rituximab, sometimes in combination with chemotherapy like chlorambucil or cyclophosphamide. Corticosteroids are also used in combination.

Plasmapheresis can be used to treat the hyperviscosity syndrome.

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