AL amyloidosis with non-amyloid forming monoclonal immunoglobulin deposition; a case mimicking AHL amyloidosis
| Autor: | Fuyuki Kametani, Masahide Yazaki, Michio Nagata, Michiyasu Hatano, Shun Manabe, Chihiro Iwasaki, Kosaku Nitta |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
medicine.medical_specialty
Pathology Amyloid 030232 urology & nephrology Case Report lcsh:RC870-923 Renal amyloidosis Lymphoplasmacytic Lymphoma Diagnosis Differential Immunoglobulin Light-chain Amyloidosis Immunoglobulin light-chain amyloidosis 03 medical and health sciences 0302 clinical medicine Internal medicine medicine AL amyloidosis Immunoglobulin heavy-and-light chain-amyloidosis Humans Aged Mass spectrometry business.industry Amyloidosis Amyloid purification medicine.disease lcsh:Diseases of the genitourinary system. Urology Nephrology 030220 oncology & carcinogenesis Monoclonal Female Immunoglobulin Light Chains Laser microdissection Immunoglobulin Heavy Chains business Immunostaining |
| Zdroj: | BMC Nephrology, Vol 19, Iss 1, Pp 1-6 (2018) BMC Nephrology |
| ISSN: | 1471-2369 |
| Popis: | Background Immunoglobulin heavy-and-light-chain amyloidosis (AHL amyloidosis) is a newly established disease entity where both the immunoglobulin heavy-chain and light-chain compose amyloid fibrils. The immunoglobulins responsible for the amyloid fibrils are generally identified by immunostaining and/or laser microdissection-liquid chromatography-tandem mass spectrometry (LMD-LC-MS/MS). However, both techniques do not biochemically differentiate immunoglobulins that formed amyloid fibrils from non-responsible immunoglobulins. Case presentation We herein report a case of 67-year-old female patient with renal amyloidosis due to lymphoplasmacytic lymphoma secreting monoclonal immunoglobulin M (IgM)-kappa. Renal immunostaining monotypically positive for IgM-kappa and LMD-LC-MS/MS identification of mu heavy-chain and kappa light-chain were consistent with the diagnosis of AHL amyloidosis. In order to confirm that both the immunoglobulin heavy-chain and light-chain were forming amyloid fibrils, we performed LC-MS/MS of renal amyloid fibrils isolated by the traditional amyloid purification method. The additional LC-MS/MS identified kappa light-chain only without any heavy-chain component. These results were suggestive that amyloid fibrils were composed by kappa light-chain only and that the mu heavy-chain identified by immunostaining and LMD-LC-MS/MS was derived from the non-specific co-deposition of monoclonal IgM-kappa. Conclusion The case was AL amyloidosis with non-amyloid forming monoclonal immunoglobulin deposition. While immunostaining and LMD-LC-MS/MS are irreplaceable techniques to classify amyloidosis, confident exclusion of the present condition should be required to diagnose AHL amyloidosis. |
| Databáze: | OpenAIRE |
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