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Perth Haematology :: Dr Steven Ward

Waldenström's Macroglobulinaemia

 

 

Waldenström’s Macrocglobulinaemia (WM)

 

WM is a disorder with features of both plasma cell disorder (like myeloma) and indolent non-Hodgkin’s Lymphoma. It is rare, occurring in approximately 5 cases per million population per year.

 

The affected cells in WM are lymphocytes and plasma cells. As in myeloma a monoclonal paraprotein is produced. In WM this is IgM in nature. IgM is a large antibody so hence the name macroglobulin.

 

 

Features:

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Slow onset of tiredness, weakness, weight loss.

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The WM cells accumulate in the marrow, blood, lymph nodes and spleen, like NHL cells.

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Features associated with the IgM paraprotein:  The IgM often causes problems in the blood from hyperviscosity (increased thickness of the plasma). The circulation becomes sluggish and this leads to headache, confusion, sleepiness, visual disturbance. Cardiac failure can occur. The Antibody can attack nerves leading to neuropathy. Renal involvement can occur.

 

Diagnosis:

Blood tests: full blood count, estimation of the paraprotein level and type; bone marrow study; CT scan to look for enlarged glands and spleen.

 

Treatment:

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Some patients have stable and slowly progressive disease without any major symptoms. No treatment would be needed in this case

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Chemotherapy: usually gentle oral therapy with chlorambucil or cyclophosphamide ± steroids are initial therapy

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More intensive therapy may be needed for younger patients, patients with a high tumour burden and hyperviscosity. CVP, CHOP, Fludarabine, rituximab, alamtuzamab (Campath) are all effective drugs.