A multiple myeloma (also known as Kahler disease, myelomatosis and plasma cell myeloma) is a malignant neoplasm of plasma cells which is characterised by (1,2):
- clonal proliferation of malignant plasma cells in the bone marrow microenvironment
- presence of monoclonal protein in the blood or urine
- associated organ dysfunction (1)
In the majority of cases, complete immunoglobulins are secreted together with an excess of Ig fragments. In up to one quarter of cases, Ig fragments only occur and the condition is referred to as light chain disease or Bence Jones myeloma.
Multiple myeloma is considered to evolve commonly from monoclonal gammopathy of undertermined clinical significance (MGUS) which develops to smouldering myeloma and finally to symptomatic myeloma (1).
Presentation is with anaemia, bone pain, skeletal destruction, pathologic fractures, or Bence Jones proteinuria and increased susceptibility to infection (3).