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Bence Jones myeloma

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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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 undetermined clinical significance (MGUS) which develops into 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).

Myeloma is the seventeenth most common cancer in the UK. In 2010, 4672 people in the UK were diagnosed with myeloma (4)

  • occurs more frequently in men and in people of African-Caribbean family origin
  • diagnosis is often delayed because the symptoms are not specific to myeloma, and this leads to significant early morbidity and mortality

Myeloma management is complex and challenging. Effective treatments have been developed over the past 15 years, and although myeloma is still incurable these treatments have led to improvements in overall survival and quality of life (4)

Note that NICE guidance now recommends the use of serum-free light chains (SFLC) rather than urinary Bence Jones protein (BJP) in the assessment of possible multiple myeloma, and studies have validated this:

SFLC replaces BJP in the British Society for Haematology/UK Myeloma Forum Guideline (5)

  • although it is noted that BJP may still be required for some clinical trials

Urine albumin:creatinine ratio along with troponin and N-terminal pro-B-type natriuretic peptide (NT-proBNP) can be a useful screening tool for detecting amyloid.

The British Society of Haematology (BSH) guidance suggests initial screening tests if suspecting multiple myeloma as (5):

  • FBC
  • Urea & creatinine
  • Calcium
  • Immunoglobulins & serum electrophoresis
  • Serum-free light chains

Reference:

  1. Palumbo A, Anderson K. Multiple myeloma. N Engl J Med. 2011;364(11):1046-60.
  2. National Cancer Institute 2011. Multiple Myeloma/Other Plasma Cell Neoplasms.
  3. Alexander DD et al. Multiple myeloma: a review of the epidemiologic literature. Int J Cancer. 2007;120
  4. NICE (February 2016).Myeloma
  5. Sive, J., Cuthill, K., Hunter, H., Kazmi, M., Pratt, G., Smith, D. and (2021), Guidelines on the diagnosis, investigation and initial treatment of myeloma: a British Society for Haematology/UK Myeloma Forum Guideline. Br. J. Haematol., 193: 245-268. https://doi.org/10.1111/bjh.1741

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