Waldenstrom's macroglobulinemia is a malignant disease of white blood cells that secrete a specific immunoglobulin (antibody; IgM).
It is also called hyperglobulinemic purpura, macroglobulinemia, Waldenstrom purpura, and Waldenstrom syndrome.
Immunoglobulin M (IgM) is one of the five classes of human antibodies produced by the body, and the largest in molecular structure. It is the first immunoglobulin the body produces when challenged by antigens and is found in circulating fluids.
IgM triggers the increased production of immunoglobulin G and the complement fixation (a mechanism) required for effective antibody response.
Increased viscosity (thickness) of the blood may result in circulatory impairment, weakness, neurologic disorders, and fatigue. Normal immunoglobulin synthesis is decreased, and the person is susceptible to infection, particularly bacterial pneumonia and septicemia.
This disease develops in patients in their 60's and70's. The cause is unknown.
Patients usually present with fatigue, mucosal or gastrointestinal bleeding, nausea, vertigo, and visual disturbances. Some patients may go into coma.
In addition to a medical history and exam, blood tests and a bone marrow biopsy may be recommended. A specific blood test called serum protein electrophoresis (SPEP) usually detects abnormally high production of IgM.
Asymptomatic patients may not require treatment until symptoms appear.
When symptomatic, those symptoms directly related to elevated IgM levels are treated by plasmapheresis alone to remove the excess IgM from the blood. (Plasmapheresis is the removal of plasma from blood by centrifugation, the reconstitution of the cellular elements in solution, and the reinfusion of this solution into the donor.)
This may require removing 4 to 6 units of plasma per day until blood viscosity returns to normal, and it needs to be continued until chemotherapy is effective.
The standard chemotherapy of Waldenstrom's macroglobulinemia consists of repeated courses of an alkylating agent, such as cyclophosphamide or chlorambucil. New agents include cladribine and fludarabine.
Autologous stem cell transplantation may be considered in younger patients with more aggressive disease.
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