*Purpose: Amyloidosis is a disorder characterized by tissue deposition of misfolded proteins that can lead to organ damage such as renal failure. There are multiple chemical types of amyloidosis. One rare type is fibrinogen alpha chain amyloidosis (AFib). We report a unique case of de novo AFib amyloidosis in a kidney transplant recipient.

*Methods: This is a 71 year old white male patient with history of coronary artery diseases on stents in 2016, HTN, Diabetes, ESRD treated with hemodialysis from 2011 to 2018. His ESRD was due to diabetes and HTN. Native kidney biopsy was not done. He had a deceased donor kidney transplantation on April 2018. There was no past medical history of connective tissue disease, malignancies, congenital kidney disease, nephrolithiasis, HIV, or hepatitis. Family History significant for sister with ESRD due diabetes. Social History was significant for 25 pack years of tobacco smoking. From January to April 2021, his serum creatinine had fluctuated from 1.28 to 1.45 mg/dL. His work-up showed negative donor specific antibodies, bland urinalysis, and negative polyomavirus and cytomegalovirus serologies. A TruGraf Blood Gene Test in June 2021 showed changes suggestive of rejection.

On August 2021, his creatinine increased to 1.71 mg/dL. He also developed lower extremity swelling and peripheral neuropathy. Serum free light chain study showed kappa light chain of 48.50 mg/dL, lambda light chain of 26.95 mg/dL, and a kappa/lambda ratio of 1.8. A transplant kidney biopsy showed amyloidosis with a positive IgM and C4d staining, but a negative for kappa and lambda chains. Mass spectrometry study showed fibrinogen alpha chain amyloidosis. Further genetic test showed point mutation with p.E545V variant.

*Results: AFib amyloidosis is caused by point mutation of fibrinogen A gene on chromosome 4 and the most common form is from the p.E545V mutation. This mutation results in hepatic synthesis of abnormal fibrinogen A molecule which later deposit in tissues. Diagnosis requires kidney biopsy and genetic testing. Afib Amyloid can lead to ESRD and can recur in transplanted kidney. However, de novo AFib amyloidosis has never been reported. Our case is the first report of this condition.

*Conclusions: Amyloidosis is usually seen as a renal disease in the native kidneys. Our case showed how this disease can occur de novo after transplantation. This case shows how hematological disorders can affect kidney transplants. These disease should be suspected in unexplained acute kidney injury in a kidney transplant with systemic symptoms such peripheral neuropathy. Prompt recognition of this disease can help management and preservation of allograft function. Treatment of AFib Amyloidosis consists of supportive care. Patient with AFib Amyloidosis with progression ESRD usually require liver or liver-kidney transplant evaluation.

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