Overview
By detailed sequence analysis and subsequent biophysical characterization of prototypic light chains, this project aims to identify sequence fingerprints in IGLV1-44 light chains leading to AL amyloidosis and POEMS syndrome. This understanding might help improve the risk stratification and early diagnosis of patients overexpressing pathologic IGLV1-44 LCs. Moreover, the development of nanobodies efficient in recognizing and stabilizing IGLV1-44 light chains which exert direct toxicity in cardiac AL amyloidosis and POEMS syndrome might form the basis for future development of therapeutic agents capable of counteracting IGLV1-44 light chain proteotoxicity.
Eligibility
Inclusion Criteria:
- Biopsy-proven diagnosis of systemic AL amyloidosis, POEMS syndrome or multiple myeloma
- Planned peripheral blood sampling +/- bone marrow aspiration
- Age \> 18 years
- Willingness to allow use of clinical data and diagnostic leftovers of clinical specimens for research purposes through signing a written informed consent.
Exclusion Criteria:
- Undefined monoclonal gammopathy or non-AL amyloidosis
- Patients fulfilling the criteria for complete hematologic response after anti-clonal therapy
- Age \<18 years
- Failure to show willingness to allow use of clinical data and diagnostic leftovers of clinical specimens for research purposes.
