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Immunoglobulin heavy chain can be amyloidogenic: morphologic characterization including immunoelectron microscopy
Hoa L Mai1, David Sheikh-Hamad, Guillermo A Herrera
1Department of Pathology, Ben Taub General Hospital and Baylor College of Medicine, Houston, TX 77030, USA.
The American Journal of Surgical Pathology
|March 27, 2003
Summary
Monoclonal immunoglobulin heavy chains can cause amyloidosis, a rare condition. This report details the first known case of IgM heavy chain amyloidosis, presenting with nephrotic syndrome.
Area of Science:
- Nephrology
- Hematology
- Pathology
Background:
- Amyloidosis is a group of diseases caused by protein misfolding and deposition.
- Heavy chain (AH) amyloidosis is a rare subtype, with few cases previously documented.
- Monoclonal immunoglobulin heavy chains are not widely recognized as amyloidogenic.
Observation:
- A 59-year-old male presented with nephrotic syndrome.
- Serum immunoelectrophoresis revealed a monoclonal IgM lambda.
- Renal biopsy confirmed amyloidosis with deposits staining positive for the mu heavy chain.
Findings:
- This case represents the first documented instance of amyloidosis caused by immunoglobulin M (IgM) heavy chains.
- The amyloid deposits were specifically identified as originating from the mu heavy chain.
- The patient's presentation of nephrotic syndrome highlights the potential renal impact.
Implications:
- This study suggests that monoclonal immunoglobulin heavy chains, including IgM, can be amyloidogenic.
- AH amyloidosis, particularly IgM-related, should be considered in the differential diagnosis of nephrotic syndrome.
- Further research is warranted to understand the mechanisms and prevalence of heavy chain amyloidosis.