Page 300 - Essential Haematology

286 / Chapter 21 Multiple myeloma and related disorders

(a) (b)
Figure 21.13 Hyperviscosity syndrome in Waldenstr ö m ’ s macroglobulinaemia. (a) The retina before

plasmapheresis shows distension of retinal vessels, particularly the veins which show bulging and constriction
(the ‘ linked sausage ’ effect) and areas of haemorrhage; (b) following plasmapheresis the vessels have returned
to normal and the areas of haemorrhage have cleared.

plasmapheresis in myeloma, Waldenstr ö m ’ s disease white cell counts. The long - term treatment depends

or hyperfibrinogenaemia; and leucopheresis or on control of the primary disease with specifi c

chemotherapy in leukaemias associated with high therapy.

SUMMARY ■ The term paraproteinaemia refers to the ■ Almost all cases of myeloma develop from

a pre - existing monoclonal gammopathy of
presence of a monoclonal immunoglobulin
undetermined signifi cance (MGUS) in
band in serum and refl ects the synthesis of
which there is low level paraprotein and no
immunoglobulin from a single clone of
evidence of tissue damage. Approximately
plasma cells.
1% of cases progress to myeloma each

■ Multiple myeloma (myelomatosis) is a

year.
tumour of plasma cells that accumulate in

the bone marrow, release a paraprotein

tissue damage in myeloma is CRAB –
and cause tissue damage. The disease
hyper c alaemia, r enal impairment, a naemia,
has a peak incidence in the seventh ■ A useful reminder for the spectrum of
decade. b one disease.