Perth Haematology :: Dr Steven Ward
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
Slow onset of
tiredness, weakness, weight loss.
The WM cells
accumulate in the marrow, blood, lymph nodes and spleen, like NHL cells.
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.
Blood tests: full
blood count, estimation of the paraprotein level and type; bone marrow study; CT
scan to look for enlarged glands and spleen.
have stable and slowly progressive disease without any major symptoms. No
treatment would be needed in this case
usually gentle oral therapy with chlorambucil or cyclophosphamide ± steroids
are initial therapy
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.