Hereditary fibrinogen A α-chain amyloidosis: phenotypic characterization of a systemic disease and the role of liver transplantation

Delayed diagnosis of fibrinogen Aα-chain amyloidosis after dual heart-kidney transplantation

“…In the cohort of 22 AFib patients [4], 52% had abnormal echocardiographic findings suggestive of amyloid cardiomyopathy (with 3/4 positive endomyocardial biopsies) and 54% had parasympathetic dysfunction with bradycardia. Prevalence of coronary atherosclerotic disease was also high (68%).…”

“…Combined hepatorenal transplantation should be considered as the first therapeutic option for patients with ESRD because of (i) the frequent recurrences of AFib amyloidosis on renal transplants, then premature transplant losses, (ii) the relatively early onset of AFib amyloidosisrelated ESRD, and (iii) the risk of systemic evolution of the disease. Thirteen cases of hepatorenal transplantation have been reported [3,4,8,9]. In the largest cohort, six of nine patients (66%) are alive with good liver and renal functions without recurrence [4].…”

“…Thirteen cases of hepatorenal transplantation have been reported [3,4,8,9]. In the largest cohort, six of nine patients (66%) are alive with good liver and renal functions without recurrence [4]. If liver transplantation is contraindicated, isolated kidney transplantation could be considered but risk of recurrence should be individually evaluated.…”

Fibrinogen amyloidosis: the clot thickens!

“…1,2 First, should rituximab be included within the regimen? Second, does the benefit of a prolonged infusion, such as the EPOCH regimen, 3 outweigh the inconvenience and cost?…”

Hereditary fibrinogen A α-chain amyloidosis: clinical phenotype and role of liver transplantation

“…2 Our experience is that AFib is, for all intents and purposes, a renal disease that is neither associated with cardiac amyloidosis nor autonomic neuropathy in any clinically meaningful way. Furthermore, we do not believe that the authors' preliminary findings justify their concluding statement encouraging preemptive solitary orthotopic liver transplantation (OLT).…”