Abstract: Objective To report and analyze relevant literature on a rare case of systemic light chain amyloidosis involving multiple systems treated in Inner Mongolia People’s Hospital, in order to improve our understanding and diagnosis of diseases. Methods Collect the diagnosis and treatment process of a rare systemic light chain amyloidosis patient with hematuria and edema as the initial symptoms who visited Inner Mongolia Autonomous Region People''s Hospital in April 2023. Results The patient was initially diagnosed with nephrotic syndrome, but the treatment effect was not satisfactory. Finally, multi-disciplinary consultation,a bone marrow biopsy was performed and Congo red staining was performed to confirm the diagnosis of amyloidosis, involving multiple systems. After chemotherapy and renal replacement therapy, the patient improved and was discharged. Conclusion The onset and clinical manifestations of amyloidosis are diverse, difficult to diagnose, and prone to misdiagnosis and missed diagnosis. Once diagnosed, chemotherapy should be administered as soon as possible. For kidney affected patients, combined dialysis is expected to restore kidney function. It is hoped that through case reports, we can improve our understanding of the disease and reduce missed diagnosis and misdiagnosis.

Key words: systemic light chain amyloidosis, multiple system damage, hematuria