De novo AL Amyloid in a Renal Allograft

Lê, Quốc Chiểu; Wood, Thomas O.; Alpers, Charles E.

doi:10.1159/000013308

Cited by 13 publications

(14 citation statements)

References 6 publications

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“…The evidence, we found in this case has supported the viewpoint [2] that amyloidosis is not a unilateral process of deposition of abnormal protein into an inert tissue, but that a complicated process, which also depends on the ability of the abnormal protein to stimulate a specific set of biologic interactions in that tissue. The rare incidence of AL amyloid deposits in the renal graft may have close relationship with the local special environment and an endogenous phagocytic/degradation process within the graft [3,4].…”

Section: Discussionsupporting

confidence: 81%

“…Since 1977, about 2,600 cases of kidney transplantation have been done in our Kidney Disease Center; however, this is the first case (0.04%) presenting with primary isolated AL amyloidosis in renal allograft. To our knowledge, only a single case with de novo AL amyloidosis in renal graft has been reported [2]. In this report, we describe an additional kidney transplant recipient who developed localized de novo AL amyloidosis in the allograft kidney 5 years after transplantation.…”

Section: Introductionmentioning

confidence: 85%

“…Primary AL amyloidosis in renal allograft is an extremely uncommon event, only a single case being described by Le QC et al [2] in 1998. However, in that case, there was no biopsy of the patient's native kidney because of the demonstration of a solitary kidney by ultrasound studies, which is very important in this diagnosis.…”

Section: Discussionmentioning

confidence: 99%

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Unusual cause of proteinuria and re-renal failure after kidney transplantation: de novo localized AL amyloidosis in renal allograft

et al. 2009

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Amyloidosis results from the deposition of amyloid proteins in organ and tissues. It can be systemic or localized. Localized primary amyloidosis in renal allograft is an extremely rare clinical condition. We describe here a 46-year-old man who developed proteinuria and re-renal failure 5 years after receiving deceased renal allograft and proved to be a case of isolated primary AL amyloidosis in renal allograft confirmed by clinical investigations and renal biopsy.

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Section: Discussionsupporting

confidence: 81%

Section: Introductionmentioning

confidence: 85%

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Unusual cause of proteinuria and re-renal failure after kidney transplantation: de novo localized AL amyloidosis in renal allograft

et al. 2009

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“…Delayed onset of proteinuria in a renal allograft can result from the development of transplant glomerulopathy, recurrent glomerular disease, diabetic nephropathy, focal segmental glomerulosclerosis, de novo membranous nephropathy, etc. De novo primary or secondary amyloidosis occurring in kidney allograft is rare with only two previously reported cases (1,2). We present four cases of de novo AL amyloidosis occurring in the renal allograft 16, 18, 28 and 31 years following kidney transplantation.…”

Section: Introductionmentioning

confidence: 99%

De novo AL Amyloidosis in the Kidney Allograft

Qian

Nasr

Fidler

et al. 2011

American Journal of Transplantation

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We report four cases of de novo amyloidosis occurring after 16, 18, 28 and 31 years following kidney transplantation. These patients presented with proteinuria and progressive allograft dysfunction. Kidney biopsy showed AL amyloidosis in all compartments of the allograft kidney. Serum immunofixation studies revealed monoclonal lambda light chains in all four cases. Bone marrow examination showed 10% plasma cells in one case, 5-10% in two cases and less than 5% in one case. Two patients died unexpectedly within 3 months and 1 year of the diagnosis of allograft AL amyloidosis. Of the remaining two, one underwent autologous stem cell transplant that resulted in complete hematologic remission. However, the patient relapsed within 2 years and also developed progressive kidney allograft failure. The patient received a second autologous stem cell transplant with complete hematologic response, followed by a second kidney transplant, which showed no evidence of amyloid at 1-year posttransplant. The remaining case was treated with prednisone and bortezomib, which has stabilized kidney function in the short term. In conclusion, this study shows that AL amyloidosis is an uncommon but important cause of late onset proteinuria in the kidney allograft that results in kidney allograft failure.

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“…Involvement of organs has often been considered as a contraindication for organ transplantation since it is a systemic disease and amyloid deposition will develop in the organ allograft. Despite these concerns, several centers have performed heart [15, 17,211 and kidney [19,25,31,451, transplantations for AL amyloidosis with good intermediate-term results in some patients. Allogeneic bone-marrow transplantation for AL amyloidosis has also been reported to lead to complete clinical recovery after 3 years [13].…”

Section: Introductionmentioning

confidence: 99%

Liver transplantation as rescue treatment in a patient with primary AL kappa amyloidosis

Nowak¹,

Westermark²,

Wernerson³

et al. 2000

Transplant International

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Although involvement of the liver is common in systemic amyloidosis, clinical manifestations of hepatic dysfunction and liver biochemical abnormalities are often absent or only mild. Here we report on a patient with primary amyloidosis and rapid development of liver failure, who was successfully treated by liver transplantation. The patient is a 61-year-old Swedish man who was admitted to the local hospital for spontaneous rupture of the spleen. Before admission, he had suffered from diffuse upper abdominal discomfort, diminished appetite, and had lost 15 kg in 6 months.Shortly after splenectomy, he developed cholestatic liver failure with moderate hepatomegaly, jaundice, ascites and hyponatremia. Over a period of 3 weeks his liver failure progressed, renal function deteriorated rapidly, and he developed encephalopathy. Liver transplantation was performed on the 35th day after splenic rupture. Histological examination revealed extensive deposits of arnyloid in the spleen and liver. N-terminal amino acid sequence analysis of the amyloid protein, purified from the patient's native liver, revealed an AL protein of kappa I-type origin. The postoperative course was uncomplicated, apart from one episode of sepsis and one course of treatment for acute rejection. He was discharged from hospital with normal liver function and good kidney function. One year after surgery, he was in good condition, with normal liver function. However, a liver biopsy taken at the same time showed de novo amyloid deposits in the grafted liver. We conclude that liver transplantation may be indicated as a life-saving procedure in rapidly progressing hepatic amyloidosis with cholestatic jaundice, although the underlying disease has not changed.

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De novo AL Amyloid in a Renal Allograft

Cited by 13 publications

References 6 publications

Unusual cause of proteinuria and re-renal failure after kidney transplantation: de novo localized AL amyloidosis in renal allograft

Unusual cause of proteinuria and re-renal failure after kidney transplantation: de novo localized AL amyloidosis in renal allograft

De novo AL Amyloidosis in the Kidney Allograft

Liver transplantation as rescue treatment in a patient with primary AL kappa amyloidosis

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