Immunoglobulin D amyloidosis: a distinct entity.
Morie A Gertz1, Francis K Buadi, Suzanne R Hayman
1Division of Hematology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA. gertz.morie@mayo.edu
Blood
|November 9, 2011
Summary
IgD monoclonal gammopathies are rare, especially in amyloidosis. This study found IgD-associated amyloidosis patients had less renal/cardiac issues but similar survival to other light-chain amyloidosis cases.
Area of Science:
- Hematology
- Oncology
- Nephrology
Background:
- IgD monoclonal gammopathies are uncommon, rarely presenting as monoclonal gammopathy of undetermined significance or multiple myeloma.
- IgD monoclonal proteins are found in approximately 1% of light-chain amyloidosis patients, often overlooked in differential diagnoses.
- Amyloidosis associated with IgD monoclonal proteins requires further investigation due to its rarity.
Purpose of the Study:
- To review the natural history of IgD-associated amyloidosis.
- To analyze clinical features and survival outcomes in patients with IgD-associated amyloidosis.
- To compare IgD-associated amyloidosis with other light-chain amyloidosis cases.
Main Methods:
- Retrospective review of 53 patients with IgD-associated amyloidosis over 41 years.
- Analysis of clinical syndromes, including renal and cardiac involvement.
- Comparison of overall survival rates with patients having other light-chain amyloidosis.
Main Results:
- IgD-associated amyloidosis patients demonstrated a lower frequency of renal and cardiac involvement.
- The clinical presentation may differ from typical light-chain amyloidosis.
- Overall survival in IgD-associated amyloidosis was comparable to other light-chain amyloidosis cases.
Conclusions:
- IgD-associated amyloidosis, though rare, presents with distinct clinical features, notably less organ involvement.
- Amyloidosis should be considered in the differential diagnosis even with IgD monoclonal proteins.
- Survival outcomes for IgD-associated amyloidosis are similar to other light-chain amyloidosis, suggesting comparable disease biology despite differing presentations.
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