Light chain deposition disease or Bence-Jones myeloma is a progressive disease characterized by plasma cell proliferation in the bone marrow and overproduction of the light chain portion of immunoglobulins (Bence Jones proteins
) in plasma and in urine .
A general examination, a detailed dermatological examination, routine blood and urine test, liver and renal function tests, random blood glucose test, urine Bence Jones proteins
, serum electrophoresis, antinuclear antibody test and an ultrasound examination of abdomen were performed in all subjects.
Bence Jones proteins
were not detected in the urine and the urinary protein electrophoresis was normal.
In 1990, it was demonstrated that increasing the concentration of sodium chloride facilitated coprecipitation of Bence Jones proteins
with human Tamm-Horsfall glycoprotein in vitro .
The investigation included a skeletal survey, bone marrowbiopsy, serum and urine protein electrophoresis, and measurements of the erythrocyte sedimentation rate and levels of [[beta].sub.2]-microglobulin, quantitative immunoglobulins, and Bence Jones proteins
in the urine.
Quantitative assays can have improved sensitivity for immunoglobulins and Bence Jones proteins
In some cases myeloma cells secrete immunoglobulins that contain only the light chains, these are secreted in the urine and are known as Bence Jones proteins
Bence Jones proteinuria, found in 60% or more of patients with multiple myeloma (MM) and less often in patients with other B-cell--derived malignancies, such as solitary plasmacytoma, Waldenstrom macroglobulinemia, and chronic lymphocytic leukemia, is serially monitored quantitatively both for its prognostic value and as an indicator of treatment response.[1-3] Renal tubular dysfunction, most likely due to a number of factors including the nephrotoxicity of Bence Jones proteins
themselves,[1,4] is a common complication of long-standing MM.
Further investigations revealed that Bence Jones proteins
were absent from the urine, and an analysis of serum immunoglobulins found no monoclonal paraproteins.
A century later, work by Korngold and Lipari (2) characterized Bence Jones proteins
and showed that they reacted with those found in myeloma.
Serum free light chains: an alternative to the urine Bence Jones proteins
screening test for monoclonal gammopathies.
A systemic workup for multiple myeloma included a bone marrow biopsy, bone scan, quantitative immunoglobulin assay, and measurements of hematocrit, white blood cells, blood urea, serum creatinine, serum electrolytes, serum calcium, and Bence Jones proteins