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Multiple Myeloma (MM) is a malignant plasma cell disorder accounting for 1% of all malignant
diseases and 10% of hematological malignancies. The annual incidence world-wide
of MM is approximately 0.4 to 5 per 100.000, with high incidence rates in North America,
Australia/New Zealand, Northern Europe, and Western Europe compared with Asian
countries. Within the United States, the incidence in African Americans is about double that
in Caucasians, whereas persons of Japanese and Chinese origin have lower rates. In the
Netherlands the annual incidence of MM is 5 per 100.000 and increases progressively with
age, the median age of diagnosis is 70 years.
MM is characterized by clonal expansion of malignant plasma cells in the bone marrow.
The myeloma plasma cell is a post-germinal centre plasma cell which has undergone somatic
hypermutation and immunoglobulin class switching. MM cells secrete a monoclonal
protein (M-protein) which can be detected in serum and/or urine. The M-protein is IgG in
50% of patients, and IgA in 30% of patients or consists of light chain (15%). In rare cases,
secretion of IgD (1%–2%), IgM (0.2%), or IgE (even less frequent), or absence of secretion
(non-secretory MM) is found.
Osteolytic bone lesions are the hallmark of MM. Other characteristic clinical features
include renal injury, anemia, hypercalcemia and immunodeficiency with recurrent
infections. These features may result directly from mass accumulation of plasma cells in
tissues (plasmacytomas) or indirectly from effects of the M-protein and/or cytokines secreted
by the plasma cells. Furthermore a high level of M-protein can cause hyperviscosity, renal
failure and neuropathy.
http://repub.eur.nl/res/pub/37640/121107_Broyl%2C%20Annemiek.pdf
http://repub.eur.nl/res/pub/37640/121107_Broyl%2C%20Annemiek.pdf