AL amyloidosis

A plasma cell disorder characterized by the aggregation and deposition of insoluble amyloid fibrils derived from misfolding of monoclonal immunoglobulin light chains usually produced by a plasma cell tumor. It usually presents as primary systemic amyloidosis (PSA) with multiple organ involvement and less frequently as primary localized amyloidosis (PLA) restricted to a single organ.

Proteinuria

Increased levels of protein in the urine.


Total: 13

                       


(per page)
PMID (PMCID)
27547738
(4982779)
 OTHER
Amyloidosis: an unusual cause of portal hypertension.
Takayasu V, Laborda LS, Bernardelli R, Pinesi HT, Silva MP, Chiavelli V, Simoes AB, Felipe-Silva A.
Autops Case Rep. 2016;6(2):9-18.
In the case reported herein, kidney involvement was also present with renal failure, massive proteinuria with monoclonal serum gammopathy, what reinforced the diagnostic possibility of primary amyloidosis.
27186381
(4858726)
 OTHER
Rare and unusual clinicopathologic presentation of renal AL amyloidosis.
Logan A, Zuppan C, Pi A, Zhang Z, Jaipaul N.
JRSM Open. 2016;7(5):2054270416640156.
Rarely, renal light chain (AL) amyloidosis may present without significant proteinuria owing to glomerular sparing and amyloid deposition confined to the vasculature and tubulointerstitium.
24040781
 FEMALE Middle Aged
An unexpected diagnosis in a renal-transplant patient with proteinuria treated with everolimus: AL amyloidosis.
Guitard J, Guilbeau-Frugier C, Roussel M, Kamar N.
Clin Nephrol. 2014;82(6):392-6.
An unexpected diagnosis in a renal-transplant patient with proteinuria treated with everolimus: AL amyloidosis.
21342449
 MIXED_SAMPLE Middle Aged
De novo AL amyloidosis in the kidney allograft.
Qian Q, Nasr SH, Fidler ME, Cornell LD, Sethi S.
Am J Transplant. 2011;11(3):606-12.
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.
19757136
 MALE Middle Aged
Unusual cause of proteinuria and re-renal failure after kidney transplantation: de novo localized AL amyloidosis in renal allograft.
Chen DJ, Jiang H, Yang H, He Q, Wang HP, Chen JH.
Int Urol Nephrol. 2010;42(2):507-11.
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.
18186230
 MALE
[Case of nephrotic syndrome due to AL-type primary amyloidosis associated with renal cell carcinoma].
Takano M, Suda N, Ichihara A, Konishi K, Sasamura H, Hayashi K, Hashiguchi A, Miyajima A, Murai M, Itoh H.
Nihon Jinzo Gakkai Shi. 2007;49(8):1014-9.
Therefore, the present patient not only represents a rare case of renal cell carcinoma associated with primary amyloidosis, but also reminds us that careful histological examination of the non-tumor region of the resected kidney is needed to evaluate the proteinuria associated with renal cell carcinoma, particularly in elderly patients.
15334239
 FEMALE Middle Aged
Axoval neuropathy as initial manifestation of primary amyloidosis: report of a case submitted to bone marrow transplantation.
Barsottini OG, Arantes A, Sigulem D, Kutner JM, Ribeiro AA, Moura LA, Hamerschlak N.
Arq Neuropsiquiatr. 2004;62(3A):725-9.
We describe the case of a 48 years-old woman with axonal neuropathy associated with proteinuria, whose final investigation resulted in diagnosis of primary amyloidosis (AL).
10401033
 MALE Middle Aged
Benign monoclonal gammopathy turning to AL amyloidosis after kidney transplantation.
Dysseleer A, Michaux L, Cosyns JP, Goffin E, Hermans C, Pirson Y.
Am J Kidney Dis. 1999;34(1):166-9.
The possible occurrence of AL amyloidosis should be kept in mind when a patient with benign monoclonal gammopathy is evaluated for organ transplantation, as well as when a transplanted patient with pre-existing monoclonal gammopathy develops new onset of proteinuria.
9619324
 MALE
Radionuclide imaging in primary amyloidosis with liver involvement.
Chen SD, Kao CH, Poon SK.
Clin Nucl Med. 1998;23(6):374-6.
A 74-year-old man with hepatomegaly, hyperglycemia, and proteinuria was diagnosed with primary amyloidosis with liver involvement, proven by biopsy.
9404936
 FEMALE Middle Aged
Allogeneic bone marrow transplantation for AL amyloidosis.
Guillaume B, Straetmans N, Jadoul M, Cosyns JP, Ferrant A.
Bone Marrow Transplant. 1997;20(10):907-8.
We report a patient with AL amyloidosis and significant proteinuria.
2509772
 FEMALE Middle Aged
Adult Fanconi syndrome in primary amyloidosis with lambda light-chain proteinuria.
Rikitake O, Sakemi T, Yoshikawa Y, Nagano Y, Watanabe T.
Jpn J Med. 1989;28(4):523-6.
To best of our knowledge, this is the first case report in which primary amyloidosis with monoclonal lambda light-chain proteinuria was accompanied by adult Fanconi syndrome.
2509772
 FEMALE Middle Aged
Adult Fanconi syndrome in primary amyloidosis with lambda light-chain proteinuria.
Rikitake O, Sakemi T, Yoshikawa Y, Nagano Y, Watanabe T.
Jpn J Med. 1989;28(4):523-6.
Adult Fanconi syndrome in primary amyloidosis with lambda light-chain proteinuria.
7453105
 MALE Middle Aged
[Primary amyloidosis with nephrotic syndrome: 5 year follow-up under polychemotherapy. A case report (author's transl)].
Emmerich B, Kircher A, Fink U, Schmid L, Rastetter J.
Klin Wochenschr. 1980;58(21):1207-13.
A patient with primary amyloidosis had infiltration of liver, bone and bone marrow and a nephrotic syndrome with massive proteinuria.