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light chain deposition disease

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https://www.readbyqxmd.com/read/27900617/new-and-evolving-concepts-regarding-the-prognosis-and-treatment-of-cardiac-amyloidosis
#1
REVIEW
Stefano Perlini, Roberta Mussinelli, Francesco Salinaro
Systemic amyloidoses are rare and proteiform diseases, caused by extracellular accumulation of insoluble misfolded fibrillar proteins. Prognosis is dictated by cardiac involvement, which is especially frequent in light chain (AL) and in transthyretin variants (ATTR, both mutated, (ATTRm), and wild-type, (ATTRwt)). Recently, ATTRwt has emerged as a potentially relevant cause of a heart failure with preserved ejection fraction (HFpEF). Cardiac amyloidosis is an archetypal example of restrictive cardiomyopathy, with signs and symptoms of global heart failure and diastolic dysfunction...
November 29, 2016: Current Heart Failure Reports
https://www.readbyqxmd.com/read/27884116/kidney-disease-in-the-elderly-biopsy-based-data-from-14-renal-centers-in-poland
#2
Agnieszka Perkowska-Ptasinska, Dominika Deborska-Materkowska, Artur Bartczak, Tomasz Stompor, Tomasz Liberek, Barbara Bullo-Piontecka, Anna Wasinska, Agnieszka Serwacka, Marian Klinger, Jolanta Chyl, Michal Kuriga, Robert Malecki, Krzysztof Marczewski, Bogdan Hryniewicz, Tadeusz Gregorczyk, Monika Wieliczko, Stanislaw Niemczyk, Olga Rostkowska, Leszek Paczek, Magdalena Durlik
BACKGROUND: Longer life expectancy is associated with an increasing prevalence of kidney disease. Aging itself may cause renal damage, but the spectrum of kidney disorders that affect elderly patients is diverse. Few studies, mostly form US, Asia and West Europe found differences in the prevalence of some types of kidney diseases between elderly and younger patients based on renal biopsy findings, with varied proportion between glomerulopathies and arterionephrosclerosis as a dominant injury found...
November 25, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27856462/clarifying-immunoglobulin-gene-usage-in-systemic-and-localized-immunoglobulin-light-chain-amyloidosis-by-mass-spectrometry
#3
Taxiarchis V Kourelis, Surendra Dasari, Jason D Theis, Marina Ramirez-Alvarado, Paul J Kurtin, Morie A Gertz, Steven R Zeldenrust, Roman M Zenka, Ahmet Dogan, Angela Dispenzieri
The goal of this study was to investigate the frequency of use of light chain variable region (IGVL) genes among patients with systemic (ALS) and localized (ALL) amyloidosis and to assess for associations between IGVL gene usage and organ tropism. We evaluated clinic charts from 821 AL patients seen at the Mayo Clinic who had bone marrow, fat pad and solid organ tissue samples typed by liquid chromatography tandem mass spectrometry (LC-MS). We identified 701 patients with ALs and 120 with ALL. Overall, we were able to identify an IGVL gene in 87 (72%) patients with ALL and 573 (82%) patients with ALS...
November 16, 2016: Blood
https://www.readbyqxmd.com/read/27825470/monoclonal-igm-related-al-amyloidosis
#4
REVIEW
Paolo Milani, Giampaolo Merlini
Monoclonal immunoglobulin M (IgM)-related light chain (AL) amyloidosis, which accounts for 5%-7% of all AL amyloidosis cases, is a distinct clinical entity that poses specific challenges to clinicians. Several studies reported that although there is a substantial overlap, the pattern of organ involvement is peculiar, with higher frequencies of lung, lymph nodes, and peripheral nervous system involvement. A recent collaborative study from three European referral centers, defined that cardiac involvement, advanced Mayo disease stage, neuropathic, and liver involvement were independent factors that had impact on survival in IgM-AL amyloidosis patients...
June 2016: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/27806176/abrupt-onset-of-refractory-heart-failure-associated-with-light-chain-amyloidosis-in-hypertrophic-cardiomyopathy
#5
Benedetta Tomberli, Francesco Cappelli, Federico Perfetto, Iacopo Olivotto
Importance: The natural history of hypertrophic cardiomyopathy (HCM) is complex and may include progressive heart failure and severe left ventricular dysfunction. When disease progression is abrupt, however, other coexisting diseases should be ruled out. This may be difficult in the case of amyloidosis, which classically mimics HCM. Results: We present an example of severe clinical deterioration in a patient with HCM due to superimposed amyloid light-chain amyloidosis...
November 2, 2016: JAMA Cardiology
https://www.readbyqxmd.com/read/27773425/unravelling-the-immunopathological-mechanisms-of-heavy-chain-deposition-disease-with-implications-for-clinical-management
#6
Frank Bridoux, Vincent Javaugue, Sébastien Bender, Fannie Leroy, Pierre Aucouturier, Céline Debiais-Delpech, Jean-Michel Goujon, Nathalie Quellard, Amélie Bonaud, Marie Clavel, Patrick Trouillas, Florent Di Meo, Jean-Marc Gombert, Jean-Paul Fermand, Arnaud Jaccard, Michel Cogné, Guy Touchard, Christophe Sirac
Randall-type heavy chain deposition disease (HCDD) is a rare disorder characterized by tissue deposition of a truncated monoclonal immunoglobulin heavy chain lacking the first constant domain. Pathophysiological mechanisms are unclear and management remains to be defined. Here we retrospectively studied 15 patients with biopsy-proven HCDD of whom 14 presented with stage 3 or higher chronic kidney disease, with nephrotic syndrome in 9. Renal lesions were characterized by nodular glomerulosclerosis, with linear peritubular and glomerular deposits of γ-heavy chain in 12 patients or α-heavy chain in 3 patients, without concurrent light chain staining...
October 20, 2016: Kidney International
https://www.readbyqxmd.com/read/27762125/bortezomib-based-chemotherapy-for-light-chain-deposition-disease
#7
Vignesh Kandakumar, Vishnu Nagalapuram, Sujaya Menon
Light chain deposition disease (LCDD) is a rare systemic disorder in which monoclonal light chains are abnormally secreted due to clonal proliferation of plasma cells and get deposited in various organs; the kidneys being the common one to be affected leading to renal failure. Advocated therapeutic options include chemotherapy with alkylating agents and steroids, High-Dose Melphalan (HDM) with Autologous Stem Cell Transplantation. Recently, Bortezomib has proven to be a novel therapeutic option in these patients when combined with dexamethasone...
August 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27756655/intracystic-hemorrhage-in-a-patient-with-pulmonary-cystic-disorder-related-to-light-chain-deposition-disease
#8
Luis Gorospe Sarasúa, Rubén-Eduardo Pacios-Blanco, Paola Arrieta, Anabelle Chinea-Rodríguez
No abstract text is available yet for this article.
October 15, 2016: Archivos de Bronconeumología
https://www.readbyqxmd.com/read/27746438/five-sequential-evaluations-of-renal-histology-in-a-patient-with-light-chain-deposition-disease
#9
Toshiharu Ueno, Koichi Kikuchi, Ryo Hazue, Koki Mise, Keiichi Sumida, Noriko Hayami, Tatsuya Suwabe, Junichi Hoshino, Naoki Sawa, Kenji Arizono, Shigeko Hara, Kenmei Takaichi, Takeshi Fujii, Kenichi Ohashi, Yoshifumi Ubara
A 58-year-old man was referred to our institution for an evaluation of nephrotic range proteinuria. Renal biopsy showed a marked expansion of the mesangial matrix and thickening of glomerular basement membrane (GBM) in periodic acid-silver methenamine (PAM). Immunofluorescence (IF) revealed strong staining for the monoclonal kappa light chain. EM demonstrated massive subendothelial and mesangial dense deposits. As a result, light chain deposition disease (LCDD) was diagnosed. Melphalan and prednisolone (MP) therapy was started, which was continued for 10 years with minimal complications...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27744229/effects-of-oxidative-post-translational-modifications-on-structural-stability-and-self-assembly-of-%C3%AE-6-immunoglobulin-light-chain
#10
Ximena Zottig, Mathieu Laporte Wolwertz, Makan Golizeh, Leanne Ohlund, Lekha Sleno, Steve Bourgault
Light chain amyloidosis (AL) originates from the deposition of immunoglobulin light chains (LCs) as amyloid fibrils in the extracellular space of vital organs. Although non-enzymatic post-translational modifications (PTMs) have been shown to contribute to protein misfolding diseases, little is known about their contributions to LC amyloidogenicity. In this study, we investigated the effects of three oxidative PTMs, carbonylation by hydroxynonenal (HNE), oxidation and nitration, on the structure, thermodynamic stability and self-assembly propensity of a LC variable domain from the λ6 germline, Wil...
October 11, 2016: Biophysical Chemistry
https://www.readbyqxmd.com/read/27728501/a-case-of-light-chain-deposition-disease-in-kidney-in-a-known-case-of-multiple-myeloma
#11
Anup Kanwar, Satish Mendonca
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27728483/unusual-case-of-light-chain-deposition-disease-kappa-restricted-in-a-case-of-multiple-myeloma
#12
S Mendonca, Arnab Choudhury
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27712634/systemic-immunoglobulin-light-chain-amyloidosis-associated-myopathy-presentation-diagnostic-pitfalls-and-outcome
#13
Eli Muchtar, Daniele Derudas, Michelle Mauermann, Teerin Liewluck, Angela Dispenzieri, Shaji K Kumar, David Dingli, Martha Q Lacy, Francis K Buadi, Suzanne R Hayman, Prashant Kapoor, Nelson Leung, Rajshekhar Chakraborty, Wilson Gonsalves, Stephen Russell, John A Lust, Yi Lin, Ronald S Go, Steven Zeldenrust, Robert A Kyle, S Vincent Rajkumar, Morie A Gertz
OBJECTIVE: To characterize the natural history of immunoglobulin light chain amyloidosis-associated myopathy and to provide guidelines for recognition. PATIENTS AND METHODS: Fifty-one patients with systemic immunoglobulin light chain amyloidosis and biopsy-confirmed muscle amyloid deposition diagnosed between January 1, 1995, and December 31, 2015, were included in this study. RESULTS: Common presenting symptoms were muscle weakness in 49 patients (96%), dysphagia in 23 (45%), myalgia in 17 (33%), macroglossia in 17 (33%), jaw claudication in 13 (25%), and hoarseness in 9 (18%)...
October 2016: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/27696268/immunoglobulin-light-chain-systemic-amyloidosis
#14
Angela Dispenzieri, Giampaolo Merlini
Immunoglobulin light chain amyloidosis (AL) is a rare, complex disease caused by misfolded free light chains produced by a usually small, indolent plasma cell clone. Effective treatments exist that can alter the natural history, provided that they are started before irreversible organ damage has occurred. The cornerstones of the management of AL amyloidosis are early diagnosis, accurate typing, appropriate risk-adapted therapy, tight follow-up, and effective supportive treatment. The suppression of the amyloidogenic light chains using the cardiac biomarkers as guide to choose chemotherapy is still the mainstay of therapy...
2016: Cancer Treatment and Research
https://www.readbyqxmd.com/read/27688084/-diabetic-nephropathy-in-a-non-diabetic-patient
#15
Olga Balafa, Georgios Liapis, Paraskevi Pavlakou, Georgios Baltatzis, Rigas Kalaitzidis, Moses Elisaf
Kimmelstiel and Wilson originally described nodular glomerulosclerosis as the pathognomonic lesion of diabetic nephropathy. Nevertheless, nodular glomerulosclerosis pattern can rarely occur in non-diabetic patients. In such cases, the differential diagnosis includes membranoproliferative glomerulonephritis, light or heavy chain deposition disease, amyloidosis, fibrillary and immunotactoid glomerulonephritis and chronic hypoxic or ischemic conditions. In cases that the above entities cannot be proven, the term idiopathic nodular glomerulosclerosis is given...
September 22, 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/27642740/-hepatic-amyloidosis-as-a-rare-differential-diagnosis-of-progressive-liver-failure
#16
Dominik Bettinger, Lisa Lutz, Michael Schultheiß, Martin Werner, Robert Thimme, Christoph Neumann-Haefelin
Primary systemic amyloidosis is a rare disorder resulting in extracellular deposition of insoluble fibrils in different organs. Liver involvement has been reported. Since hepatic amyloidosis often presents clinically asymptomatic without specific laboratory or imaging hallmarks, diagnosis is challenging. However, cases of progressive hepatic failure due to liver amyloidosis have been reported. A 63 year old man presented with newly diagnosed ascites to our department. The patient reported occasional alcohol consumption...
September 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27634125/al-light-chain-cardiac-amyloidosis-a-review-of-diagnosis-and-therapy
#17
REVIEW
Rodney H Falk, Kevin M Alexander, Ronglih Liao, Sharmila Dorbala
The amyloidoses are a group of protein-folding disorders in which ≥1 organ is infiltrated by proteinaceous deposits known as amyloid. The deposits are derived from 1 of several amyloidogenic precursor proteins, and the prognosis of the disease is determined both by the organ(s) involved and the type of amyloid. Amyloid involvement of the heart (cardiac amyloidosis) carries the worst prognosis of any involved organ, and light-chain (AL) amyloidosis is the most serious form of the disease. The last decade has seen considerable progress in understanding the amyloidoses...
September 20, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/27629972/successful-treatment-of-amyloid-light-chain-amyloidosis-in-a-charcot-marie-tooth-disease-patient-with-lenalidomide-cyclophosphamide-and-dexamethasone
#18
Yoshitaka Kikukawa, Hiroyuki Hata, Mitsuharu Ueda, Taro Yamashita, Singo Nasu, Kazuhiko Ide, Shikiko Ueno, Yukio Ando, Hiroaki Mitsuya, Yutaka Okuno
A 70-year-old woman with Charcot-Marie-Tooth disease (CMT) suffered from nephrotic syndrome and a renal biopsy revealed non-AA amyloid depositions that contained immunoglobulin light chain λ. Her serum λ free LC was elevated to 80.8 mg/L and she was diagnosed with primary amyloid light-chain (AL) amyloidosis. She was subsequently treated with lenalidomide, cyclophosphamide, and dexamethasone (RCD). After 14 cycles of RCD, she achieved complete remission. Her serum albumin levels gradually normalized to 3...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27624800/localized-primary-amyloidosis-of-the-breast-a-case-report-and-review-of-the-literature
#19
Wakako Tsuji, Eiji Takeuchi, Satoshi Oka, Taro Yamashita, Fumiaki Yotsumoto
BACKGROUND: Primary amyloidosis of the breast is an unusual benign disease that mostly occurs in postmenopausal elderly women. Amyloidosis is the deposition of amorphous protein within tissues. Breast biopsy is necessary to make a definite diagnosis in order to avoid unnecessary surgical methods. Localized primary amyloidosis of the breast has a good prognosis. However, secondary amyloidosis is a systemic disease and has a poor prognosis. CASE PRESENTATION: We report the case of a 77-year-old female with primary amyloidosis of the breast...
September 13, 2016: BMC Surgery
https://www.readbyqxmd.com/read/27574973/effect-of-systemic-iron-overload-and-a-chelation-therapy-in-a-mouse-model-of-the-neurodegenerative-disease-hereditary-ferritinopathy
#20
Holly J Garringer, Jose M Irimia, Wei Li, Charles B Goodwin, Briana Richine, Anthony Acton, Rebecca J Chan, Munro Peacock, Barry B Muhoberac, Bernardino Ghetti, Ruben Vidal
Mutations in the ferritin light chain (FTL) gene cause the neurodegenerative disease neuroferritinopathy or hereditary ferritinopathy (HF). HF is characterized by a severe movement disorder and by the presence of nuclear and cytoplasmic iron-containing ferritin inclusion bodies (IBs) in glia and neurons throughout the central nervous system (CNS) and in tissues of multiple organ systems. Herein, using primary mouse embryonic fibroblasts from a mouse model of HF, we show significant intracellular accumulation of ferritin and an increase in susceptibility to oxidative damage when cells are exposed to iron...
2016: PloS One
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