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.

Nephrotic syndrome

Nephrotic syndrome is a collection of findings resulting from glomerular dysfunction with an increase in glomerular capillary wall permeability associated with pronounced proteinuria. Nephrotic syndrome refers to the constellation of clinical findings that result from severe renal loss of protein, with Proteinuria and hypoalbuminemia, edema, and hyperlipidemia.


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(per page)
PMID (PMCID)
30548910
 OTHER
Kidney embolization induces prompt organ response in a 86-year-old patient with MGRS-related AL-amyloidosis.
Kuchlin S, Duffner J, Scheubeck S, Schoeller K, Maruschke L, Seidl M, Engelhardt M, Walz G, Prager EP, Waldschmidt JM.
Hemodial Int. 2019;23(2):E59-E64.
Here, we report on the case of a severely frail 86-year-old patient suffering from monoclonal gammopathy of renal significance (MGRS)-associated AL-amyloidosis with a diuretic-refractory nephrotic syndrome.
30886753
 OTHER
Radiologically Guided Renal Artery Embolization with an Amplatzer Vascular Plug as a Rescue Therapy for Refractory Nephrotic Syndrome in AL-Amyloidosis.
Zust IS, Raz HR, Burkhalter F.
Case Rep Nephrol. 2019;2019:5469712.
We report the case of a 63-year-old patient with severe nephrotic syndrome due to primary renal AL-amyloidosis with well-preserved renal function at first presentation.
30886753
 OTHER
Radiologically Guided Renal Artery Embolization with an Amplatzer Vascular Plug as a Rescue Therapy for Refractory Nephrotic Syndrome in AL-Amyloidosis.
Zust IS, Raz HR, Burkhalter F.
Case Rep Nephrol. 2019;2019:5469712.
Nephrotic syndrome is common in immunoglobulin light-chain (AL) amyloidosis and successful therapy may pose a challenge.
30886753
 OTHER
Radiologically Guided Renal Artery Embolization with an Amplatzer Vascular Plug as a Rescue Therapy for Refractory Nephrotic Syndrome in AL-Amyloidosis.
Zust IS, Raz HR, Burkhalter F.
Case Rep Nephrol. 2019;2019:5469712.
Radiologically Guided Renal Artery Embolization with an Amplatzer Vascular Plug as a Rescue Therapy for Refractory Nephrotic Syndrome in AL-Amyloidosis.
26965175
 MALE Adult
Acute liver failure due to primary amyloidosis in a nephrotic syndrome: a swiftly progressive course.
Cardoso BA, Leal R, Sa H, Campos M.
BMJ Case Rep. 2016;2016:.
We report a case of a 39-year-old man admitted to our nephrology department with an unusual presentation of primary amyloidosis with nephrotic syndrome and acute liver failure, complicated by obstructive cholestasis resulting in death 28197months after diagnosis.
26965175
 MALE Adult
Acute liver failure due to primary amyloidosis in a nephrotic syndrome: a swiftly progressive course.
Cardoso BA, Leal R, Sa H, Campos M.
BMJ Case Rep. 2016;2016:.
Acute liver failure due to primary amyloidosis in a nephrotic syndrome: a swiftly progressive course.
26965175
 MALE Adult
Acute liver failure due to primary amyloidosis in a nephrotic syndrome: a swiftly progressive course.
Cardoso BA, Leal R, Sa H, Campos M.
BMJ Case Rep. 2016;2016:.
The most common organs affected by AL amyloidosis are the kidney, presenting with nephrotic syndrome and/or progressive renal dysfunction, and the heart, with restrictive cardiomyopathy.
25672860
 MALE
[Nephrotic syndrome might be an inhibitor of the endovascular recanalization in a patient with acute ischemic stroke].
Nakanishi T, Watanabe M, Nakajima M, Nojiri N, Oda A, Ando Y.
Rinsho Shinkeigaku. 2015;55(1):18-22.
Renal biopsy confirmed nephrotic syndrome due to AL amyloidosis.
24681937
 FEMALE Middle Aged
[Primary systemic AL amyloidosis with remarkable calcification in the spleen].
Uchiyama K, Tsukada N, Miyazaki K, Abe Y, Sekine R, Nakagawa Y, Suzuki K.
Rinsho Ketsueki. 2014;55(3):334-9.
She had developed nephrotic syndrome and been diagnosed with renal amyloid light-chain amyloidosis in 2010.
24452148
 MALE Middle Aged
[Rapidly progressive AL amyloidosis in a patient with relapsed multiple myeloma after achieving a complete response to tandem autologous PBSCT].
Yagi H, Ozaki S, Sekimoto E, Shibata H, Shigekiyo T, Satake N.
Rinsho Ketsueki. 2013;54(12):2171-6.
In 2012, he developed edema of the lower legs and pleural effusion, and was diagnosed as having nephrotic syndrome and heart failure due to AL amyloidosis.
29497519
(5783203)
 OTHER
Renal ablation using bilateral ureteral ligation for nephrotic syndrome due to renal amyloidosis.
Keddis MT, Stegall MD, Textor SC.
Clin Kidney J. 2012;5(2):153-154.
Nephrotic syndrome is common in immunoglobulin light chain amyloidosis (AL).
21995711
(3206418)
 MALE Middle Aged
Concurrent nephrotic syndrome and acute renal failure caused by chronic lymphocytic leukemia (CLL): a case report and literature review.
Dou X, Hu H, Ju Y, Liu Y, Kang K, Zhou S, Chen W.
Diagn Pathol. 2011;6:99.
To our knowledge, this is the first case of CLL patient showing the nephrotic syndrome and acute renal failure caused by AL amyloidosis and neoplastic infiltration.
25984105
(4421635)
 OTHER
Spontaneous bilateral kidney rupture during autologous stem cell transplantation in a patient affected by amyloidosis.
Ferrannini M, Vischini G, De Angelis G, Giannakakis K, Arcese W.
NDT Plus. 2011;4(1):56-9.
A 46-year-old white woman, affected by nephrotic syndrome, was diagnosed as AL amyloidosis by renal biopsy.
20088306
 MALE Adult
Primary amyloidosis of the kidney.
Shafique S, Wetmore J, Almehmi A.
W V Med J. 2010;106(1):22-4.
The kidney is frequently involved in AL amyloidosis; nephrotic syndrome and/or end stage renal disease (ESRD) occur at presentation in about two-thirds of the patients.
20017442
 FEMALE Adult
[Complete remission of nephrotic syndrome and improvement of renal function in a patient with light chain deposition disease following high dose chemotherapy with transplantation of autologous haematopoietic stem cells. A case study and review of literature].
Adam Z, Krejci M, Pour L, Stepankova S, Cermakova Z, Voska L, Teplan V, Krivanova A, Hajek R, Mayer J.
Vnitr Lek. 2009;55(11):1089-96.
This has been previously described in patients with AL-amyloidosis, and nephrotic syndrome who were treated with high dose melphalan.
20049380
 MALE Middle Aged
[Effectiveness of dietetic treatment in nephrotic syndrome].
Calleja Fernandez A, Lopez Gomez JJ, Vidal Casariego A, Cano Rodriguez I, Ballesteros Pomar MD.
Nutr Hosp. 2009;24(6):744-7.
We present the case of a male patient suffering from a primary amyloidosis and a nephrotic syndrome who came to dietotherapy clinic.
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.
[Case of nephrotic syndrome due to AL-type primary amyloidosis associated with renal cell carcinoma].
16632032
 FEMALE Middle Aged
AH amyloidosis associated with lymphoplasmacytic lymphoma secreting a monoclonal gamma heavy chain carrying an unusual truncated D segment.
Gono T, Yazaki M, Fushimi T, Suzuki T, Uehara T, Sano K, Kametani F, Ito N, Matsushita M, Nakamura S, Hoshii Y, Matsuda M, Ikeda S.
Am J Kidney Dis. 2006;47(5):908-14.
Although AH amyloidosis is caused mainly by plasma cell dyscrasia, as in AL amyloidosis, we report a 61-year-old patient who presented with nephrotic syndrome caused by AH amyloidosis associated with lymphoplasmacytic lymphoma.
17130952
 FEMALE Middle Aged
[White blood cell lysis syndrome after autologous peripheral blood stem cell transplantation in the treatment of renal AL amyloidosis. Case report].
Gatica A, Bertin P, Tagle R.
Rev Med Chil. 2006;134(6):763-6.
We report a 59 year-old female with a nephrotic syndrome due to primary amyloidosis successfully treated autologous stem cell transplantation who developed acute renal failure caused by white blood cell lysis syndrome.
16082995
 MALE Middle Aged
Primary amyloidosis (AL) as a cause of nephrotic syndrome.
Bataille Y, Bovy C, Lancellotti P, Melchior V, Delbecque K, Beguin Y, Krzesinski JM.
Acta Clin Belg. 2005;60(2):94-7.
By illustrating the case of a patient whose AL amyloidosis was detected after presenting a nephrotic syndrome, the characteristics of the disease are reviewed as well as diagnostic criteria and current available therapeutics.