These conditions are dyscrasias, proliferation of mature B cells with
secondary clonal expansion. There is often dissociated production
of light and heavy Ig chains. Multiple myeloma is the best known,
and characteristically has multiple bony lesions and Bence-Jones proteinuria
(Ig light chains). Plasmacytoma (plasma cell myeloma) is an isolated
lesion of the same type in bone or soft tissue (although rarely in lymph
nodes). An isolated bone finding could be early MM, but may also
be stable for decades. The soft tissue leions can usually be treated
locally. Heavy chain disease (also known as Mediteranean lymphoma
is common in undernourished populations and is characterized by Ig heavy
chain secretion. Primary or immune amyloidosis is often secondary
to plasma cell secretion of light (usually lambda) chains. Lymphoblastic
lymphoma (also known as small lymphocytic lymphoma with plasma cell differentiation)
results in Waldenstrom macroglobulinemia. The production of excessive
IgM in this syndrome results in a hyperviscosity syndrome. Cold agglutinins
may also result in hemolysis. MGUS is a monoclonal gammapathy which
is not associated with a dyscrasia, but in about 20% of cases, some dyscrasia
develops over the succeeding two decades.
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| Heme-Onc |
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