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Light chain multiple myeloma is diagnosed in patients who have: a) the criteria for diagnosis of multiple myeloma except having a serum free light chain ratio outside the normal range of 0.26 to 1.65 without evidence of an intact immunoglobulin or free heavy chain; or b) an extreme free light chain ratio, i.e. outside the range of 0.02 to 100 ...
This is due to an outgrowth of a single type of plasma cell that forms the plasmacytoma and produces a single type of antibody. The plasma cells are said to be monoclonal and the excessively produced antibody is known as monoclonal protein or paraprotein. [6] Paraproteins are present in 60% of SPB and less than 25% of extramedullary ...
Light chain deposition disease can affect any organ. [3] Renal involvement is always present and can be identified by microscopic hematuria and proteinuria.Due to the gradual buildup of light chains from plasma filtration, renal function rapidly declines in the majority of patients with LCDD as either acute tubulointerstitial nephritis or rapidly progressing glomerulonephritis.
Serum free light chain measurement is positive in 100% of cases, evidence that the underlying monoclonal plasma cells secrete free light chains as well as heavy chains. [2] [4] HCDD is associated with multiple myeloma in 29% of cases. [2] Deposition of complement protein C3 (and sometimes Cq1) may also be seen in HCDD. [2]
Smouldering myeloma is a disease classified as intermediate in a spectrum of step-wise progressive diseases termed plasma cell dyscrasias.In this spectrum of diseases, a clone of plasma cells secreting monoclonal paraprotein (also termed myeloma protein or M protein) causes the relatively benign disease of monoclonal gammopathy of undetermined significance.
Monoclonal gammopathy of renal significance. Monoclonal gammopathy of renal significance (MGRS) are a group of kidney disorders that present with kidney damage due to nephrotoxic monoclonal immunoglobulins (M proteins) secreted by clonal plasma cells or B cells. By definition, people with MGRS do not meet criteria for multiple myeloma or other ...
Plasma cell dyscrasia. Plasma cell dyscrasias are a group of monoclonal gammopathies in which normal plasma cells in the bone marrow and soft tissues become altered. POEMS syndrome is often associated with an IgA or IgG lambda limited plasma cell dysfunction. On iliac crest biopsies, patients with POEMS syndrome often have few monoclonal plasma ...
Plasma cell leukemia (PCL) is a plasma cell dyscrasia, i.e. a disease involving the malignant degeneration of a subtype of white blood cells called plasma cells. It is the terminal stage and most aggressive form of these dyscrasias, constituting 2% to 4% of all cases of plasma cell malignancies. PCL may present as primary plasma cell leukemia ...