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Light chain amyloidosis

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https://www.readbyqxmd.com/read/28334621/sequential-cyclophosphamide-bortezomib-dexamethasone-unmasks-the-harmful-cardiac-effect-of-dexamethasone-in-primary-light-chain-cardiac-amyloidosis
#1
Fabien Le Bras, Valerie Molinier-Frenkel, Aziz Guellich, Jehan Dupuis, Karim Belhadj, Soulef Guendouz, Karima Ayad, Magali Colombat, Nicole Benhaiem, Claire Marie Tissot, Anne Hulin, Arnaud Jaccard, Thibaud Damy
Chemotherapy combining cyclophosphamide, bortezomib and dexamethasone is widely used in light-chain amyloidosis. The benefit is limited in patients with cardiac amyloidosis mainly because of adverse cardiac events. Retrospective analysis of our cohort showed that 39 patients died with 42% during the first month. A new escalation-sequential regimen was set to improve the outcomes. Nine newly-diagnosed patients were prospectively treated with close monitoring of serum N-terminal pro-brain natriuretic peptide, troponin-T and free light chains...
March 20, 2017: European Journal of Cancer
https://www.readbyqxmd.com/read/28326212/a-case-of-conjunctival-amyloidosis-with-repeated-subconjunctival-hemorrhage
#2
Takaaki Ando, Toshiyuki Oshitari, Mamiko Saito, Ayako Tawada, Takayuki Baba, Jiro Yotsukura, Shuichi Yamamoto
Conjunctival amyloidosis is a very rare disease, and its presence may be a sign of systemic amyloidosis. We present our ocular and systemic findings in a patient with conjunctival amyloidosis. A 43-year-old man had repeated subconjunctival hemorrhages (SCHs) for two years and was referred to the Chiba University Hospital. He had comprehensive ophthalmological and systemic examinations to determine the cause of the SCHs. His visual acuities were 1.2 OU, and the intraocular pressures were 13-14 mmHg OU. Magnetic resonance imaging was normal...
2017: Case Reports in Ophthalmological Medicine
https://www.readbyqxmd.com/read/28314084/treatment-patterns-and-outcome-following-initial-relapse-or-refractory-disease-in-patients-with-systemic-light-chain-amyloidosis
#3
Nidhi Tandon, Surbhi Sidana, Morie A Gertz, Angela Dispenzieri, Martha Q Lacy, Francis K Buadi, David Dingli, Amie L Fonder, Miriam A Hobbs, Suzanne R Hayman, Wilson I Gonsalves, Yi Lisa Hwa, Prashant Kapoor, Robert A Kyle, Nelson Leung, Ronald S Go, John A Lust, Stephen J Russell, Steven R Zeldenrust, S Vincent Rajkumar, Shaji K Kumar
We analyzed the outcomes following initial relapse or refractory disease in systemic light chain amyloidosis (AL) and the impact of type of therapy employed.A total of 1327 patients with AL seen at Mayo Clinic within 90 days of diagnosis, between 2006 and 2015, were reviewed. The study included 366 patients experiencing a documented hematological or organ relapse or refractory disease requiring start of second line therapy. Overall survival (OS) and time to next treatment (TTNT) were calculated from start of second line treatment...
March 17, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28298286/echo-parameters-for-differential-diagnosis-in-cardiac-amyloidosis-a-head-to-head-comparison-of-deformation-and-nondeformation-parameters
#4
Efstathios D Pagourelias, Oana Mirea, Jürgen Duchenne, Johan Van Cleemput, Michel Delforge, Jan Bogaert, Tatyana Kuznetsova, Jens-Uwe Voigt
BACKGROUND: A plethora of echo parameters has been suggested for distinguishing cardiac amyloidosis (CA) from other causes of myocardial thickening with, however, scarce data on their head-to-head comparison. This study aimed at comparing the diagnostic accuracy of various deformation and conventional echo parameters in differentiating CA from other hypertrophic substrates, especially in the gray zone of mild hypertrophy (maximum wall thickness ≤16 mm) or normal ejection fraction (EF)...
March 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/28295502/assessment-of-renal-response-with-urinary-exosomes-in-patients-with-al-amyloidosis-a-proof-of-concept
#5
Marina Ramirez-Alvarado, David R Barnidge, David L Murray, Angela Dispenzieri, Marta Marin-Argany, Christopher J Dick, Shawna A Cooper, Samih H Nasr, Christopher J Ward, Surendra Dasari, Víctor H Jiménez-Zepeda, Nelson Leung
Immunoglobulin light chain (AL) amyloidosis is a fatal complication of B-cell proliferation secondary to deposition of amyloid fibrils in various organs. Urinary exosomes (UEX) are the smallest of the microvesicles excreted in the urine. Previously, we found UEX of patients with AL amyloidosis contained immunoglobulin light chain (LC) oligomers that patients with multiple myeloma did not have. To further explore the role of the LC oligomers, UEX was isolated from an AL amyloidosis patient with progressive renal disease despite achieving a complete response...
March 10, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28293126/the-risk-of-renal-disease-is-increased-in-lambda-myeloma-with-bone-marrow-amyloid-deposits
#6
Piotr Kozlowski, Scott Montgomery, Rahel Befekadu, Victoria Hahn-Strömberg
BACKGROUND: Light chain amyloidosis (AL) is a rare deposition disease and is present in 10-15% of patients with myeloma (MM). In contrast to symptomatic AL in MM, presence of bone marrow (BM) amyloid deposits (AD) in MM is not connected to kidney damage. Renal AD but not BM-AD occur mostly in MM with lambda paraprotein (lambda MM). METHODS: We investigated amyloid presence in BM clots taken at diagnosis in 84 patients with symptomatic MM and compared disease characteristics in MM with kappa paraprotein (kappa MM)/lambda MM with and without BM-AD...
2017: Journal of Blood Medicine
https://www.readbyqxmd.com/read/28290973/total-artificial-heart-implantation-as-a-bridge-to-heart-transplantation-in-an-active-duty-service-member-with-amyloid-cardiomyopathy
#7
Michael S Scully, Dylan E Wessman, James M McKee, Gregory M Francisco, Keshav R Nayak, Jon A Kobashigawa
INTRODUCTION: Cardiac involvement by light-chain (AL) amyloid occurs in up to 50% of patients with primary AL amyloidosis. The prognosis of amyloid heart disease is poor with 1-year survival rates of 35 to 40%. Historically, heart transplantation was considered controversial for patients with AL amyloid cardiomyopathy (CM) given the systemic nature of the disease and poor survival. We present a case report of an active duty service member diagnosed with advanced cardiac amyloid who underwent total artificial heart transplant as a bridge to heart transplant and eventual autologous stem cell transplant...
March 2017: Military Medicine
https://www.readbyqxmd.com/read/28286788/primary-hepatic-amyloidosis-presenting-as-acute-on-chronic-liver-failure
#8
Madhumita Premkumar, Devaraja Rangegowda, Tanmay Vyas, Anand Kulkarni, Shrruti Grover, Rakhi Mahiwall, Shiv Kumar Sarin
Systemic amyloidosis of amyloid light chain associated protein (AL), also called primary amyloidosis, frequently involves the liver, but rarely causes clinically apparent liver disease. The more common presentation is with acute renal failure. Hepatomegaly and mild elevation of alkaline phosphatase are the most common clinical and biochemical findings, respectively. We report a case of systemic amyloidosis of AL that clinically presented as acute-on-chronic liver failure and resulted in a fatal clinical course in a 56-year-old man...
2017: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28281017/heart-transplantation-in-cardiac-amyloidosis
#9
REVIEW
Matthew Sousa, Gregory Monohan, Navin Rajagopalan, Alla Grigorian, Maya Guglin
"Cardiac amyloidosis" is the term commonly used to reflect the deposition of abnormal protein amyloid in the heart. This process can result from several different forms, most commonly from light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis, which in turn can represent wild-type (ATTRwt) or genetic form. Regardless of the origin, cardiac involvement is usually associated with poor prognosis, especially in AL amyloidosis. Although several treatment options, including chemotherapy, exist for different forms of the disease, cardiac transplantation is increasingly considered...
March 9, 2017: Heart Failure Reviews
https://www.readbyqxmd.com/read/28279033/-analysis-of-clinical-characteristics-and-outcome-of-patients-with-very-high-risk-primary-immunoglobulin-light-chain-amyloidosis
#10
J Feng, X F Huang, C L Zhang, K N Shen, C L Zhang, J Sun, Z Tian, X X Cao, L Zhang, D B Zhou, J Li
Objective: To evaluate the clinical characteristics and outcomes of very high risk patients with primary immunoglobulin light-chain amyloidosis (pAL) at a single center in China. Method: Clinical data, treatment and outcome of 205 pAL patients in Peking Union Medical College Hospital from January 2009 to February 2016 were retrospectively analyzed. A 'very high risk' group includes patients with Mayo 2004 stage Ⅲb and Mayo 2012 stage 4. Results: Of 205 patients, 34 (16.6%) were defined as very high risk pAL patients...
February 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28278302/neuropathy-and-efficacy-of-once-weekly-subcutaneous-bortezomib-in-multiple-myeloma-and-light-chain-al-amyloidosis
#11
Surbhi Sidana, Mayur Narkhede, Paul Elson, Debbie Hastings, Beth Faiman, Jason Valent, Christy Samaras, Kimberly Hamilton, Hien K Liu, Mitchell R Smith, Frederic J Reu
INTRODUCTION: Randomized studies have shown that bortezomib (BTZ) can be given weekly via intravenous (IV) route or twice weekly via subcutaneous (SC) route with lower neuropathy risk and no loss of anti-myeloma efficacy compared to original standard IV twice weekly schedule. Weekly SC should therefore yield the best therapeutic index and is widely used but has not been compared to established administration schedules in the context of a clinical trial. METHODS: Comprehensive electronic medical record review was done for disease control and neuropathy symptoms of 344 consecutive patients who received their first BTZ-containing regimen for myeloma or AL amyloidosis before or after we changed to SC weekly in December 2010...
2017: PloS One
https://www.readbyqxmd.com/read/28277869/disease-burden-of-systemic-light-chain-al-amyloidosis-a-systematic-literature-review
#12
Huamao Mark Lin, Xin Gao, Catherine E Cooke, Deborah Berg, Richard Labotka, Douglas V Faller, Brian Seal, Parameswaran Hari
A systematic literature review on AL amyloidosis was conducted in order to understand the disease burden, and identify unmet medical needs and knowledge gaps. MEDLINE, EMBASE and COCHRANE databases were searched for English language studies published in the last 10 years using search terms that focused on the clinical, economic, and patient-reported outcome (PRO) aspects of AL amyloidosis. There was a low yield of articles in the economic and PRO categories and additional searches were conducted in clinical conference proceedings, and using Google and Google Scholar...
February 21, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28271734/overuse-of-organ-biopsies-in-immunoglobulin-light-chain-al-amyloidosis-the-consequence-of-failure-of-early-recognition
#13
Eli Muchtar, Angela Dispenzieri, Martha Q Lacy, Francis K Buadi, Prashant Kapoor, Suzanne R Hayman, Wilson Gonsalves, Rahma Warsame, Taxiarchis V Kourelis, Rajshekhar Chakraborty, Stephen Russell, John A Lust, Yi Lin, Ronald S Go, Steven Zeldenrust, S Vincent Rajkumar, David Dingli, Nelson Leung, Robert A Kyle, Shaji K Kumar, Morie A Gertz
INTRODUCTION: The diagnosis of amyloidosis requires histological confirmation of Congo-red (CR) deposits. The tissue source is preferably fat aspiration and/or bone marrow biopsy, but at times organ biopsy is required. METHODS: We studied 612 patients with systemic immunoglobulin light chain amyloidosis to characterize the tissues used to establish the diagnosis. RESULTS: The median number of tissue samples was 3. 95% of bone marrow (BM) biopsies were stained for CR, while 79% of patients had fat aspiration CR-stained...
March 8, 2017: Annals of Medicine
https://www.readbyqxmd.com/read/28265790/value-of-tissue-doppler-derived-tei-index-and-two-dimensional-speckle-tracking-imaging-derived-longitudinal-strain-on-predicting-outcome-of-patients-with-light-chain-cardiac-amyloidosis
#14
Dan Liu, Kai Hu, Sebastian Herrmann, Maja Cikes, Georg Ertl, Frank Weidemann, Stefan Störk, Peter Nordbeck
Prognosis of patients with light-chain cardiac amyloidosis (AL-CA) is poor. Speckle tracking imaging (STI) derived longitudinal deformation parameters and Doppler-derived left ventricular (LV) Tei index are valuable predictors of outcome in patients with AL-CA. We estimated the prognostic utility of Tei index and deformation parameters in 58 comprehensively phenotyped patients with AL-CA after a median follow-up of 365 days (quartiles 121, 365 days). The primary end point was all-cause mortality. 19 (33%) patients died during follow-up...
March 6, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/28261692/immunoglobulin-light-chains-form-an-extensive-and-highly-ordered-fibril-involving-the-n-and-c-termini
#15
Dennis W Piehl, Luis M Blancas-Mejía, Jonathan S Wall, Stephen J Kennel, Marina Ramirez-Alvarado, Chad M Rienstra
Light-chain (AL)-associated amyloidosis is a systemic disorder involving the formation and deposition of immunoglobulin AL fibrils in various bodily organs. One severe instance of AL disease is exhibited by the patient-derived variable domain (VL) of the light chain AL-09, a 108 amino acid residue protein containing seven mutations relative to the corresponding germline protein, κI O18/O8 VL. Previous work has demonstrated that the thermodynamic stability of native AL-09 VL is greatly lowered by two of these mutations, Y87H and N34I, whereas a third mutation, K42Q, further increases the kinetics of fibril formation...
February 28, 2017: ACS Omega
https://www.readbyqxmd.com/read/28247530/relapsing-bullous-amyloidosis-of-the-oral-mucosa-and-acquired-cutis-laxa-in-a-patient-with-multiple-myeloma-a-rare-triple-association
#16
J Gonzalez-Ramos, C Garrido-Gutiérrez, Y González-Silva, L Yébenes-Gregorio, M Beato-Merino, C Vidaurrázaga-Arcaya, P Herranz-Pinto
It is well known that primary systemic amyloidosis [light chain (AL) amyloidosis] is associated with hidden dyscrasia or multiple myeloma. Acquired cutis laxa (cutis laxa acquisita; CLA) has also been described in patients with plasma cell dyscrasias, including multiple myeloma. We report a case in which haemorrhagic oral bullae were the first sign of an undiagnosed primary systemic amyloidosis related to multiple myeloma IgG-λ and previously diagnosed CLA. There is only one report in literature of this rare triple association; however, in that case the patient did not have oral mucosal involvement or bullous amyloidosis...
March 1, 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/28219562/isolated-pericardial-infiltration-without-myocardial-involvement-in%C3%A2-light-chain-related-amyloidosis
#17
Sophie Georgin-Lavialle, Aziz Guellich, Jean-François Deux, David Buob, Thibaud Damy
Light-chain-related amyloidosis is a systemic disease characterized by continuous accumulation of insoluble fibrillar proteins in different organs. Cardiac involvement is frequent in this condition. However, atypical presentations and unusual amyloid deposits localization may be encountered making the diagnosis challenging. We present here a case of a light-chain-related pericardial amyloidosis without evidence of myocardial involvement and emphasize the difficulty and importance of amyloidosis typing before starting treatment...
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28202864/clinical-presentation-of-tubulointerstitial-nephritis-caused-by-amyloid-light-chain-amyloidosis-in-a-patient-with-sj%C3%A3-gren-s-syndrome
#18
Reiko Inoue, Yoshihide Fujigaki, Kana Kobayashi, Yoshifuru Tamura, Tatsuru Ota, Shigeru Shibata, Tsuyoshi Ishida, Fukuo Kondo, Yutaka Yamaguchi, Shunya Uchida
We report a 70-year-old woman with Sjögren's syndrome who had severe renal dysfunction with mild proteinuria and elevated urinary low-molecular-weight proteins. Based on these clinical presentations, interstitial nephritis due to Sjögren's syndrome was strongly suspected. Unexpectedly, renal pathology revealed amyloid light-chain (AL) lambda-type depositions predominantly in the vasculatures with severe tubulointerstitial damage. Concentrated urine immunofixation was positive for Bence Jones lambda-type monoclonal proteins...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28188192/multiple-myeloma-version-3-2017-nccn-clinical-practice-guidelines-in-oncology
#19
Shaji K Kumar, Natalie S Callander, Melissa Alsina, Djordje Atanackovic, J Sybil Biermann, Jason C Chandler, Caitlin Costello, Matthew Faiman, Henry C Fung, Cristina Gasparetto, Kelly Godby, Craig Hofmeister, Leona Holmberg, Sarah Holstein, Carol Ann Huff, Adetola Kassim, Michaela Liedtke, Thomas Martin, James Omel, Noopur Raje, Frederic J Reu, Seema Singhal, George Somlo, Keith Stockerl-Goldstein, Steven P Treon, Donna Weber, Joachim Yahalom, Dorothy A Shead, Rashmi Kumar
Multiple myeloma (MM) is caused by the neoplastic proliferation of plasma cells. These neoplastic plasma cells proliferate and produce monoclonal immunoglobulin in the bone marrow causing skeletal damage, a hallmark of multiple myeloma. Other MM-related complications include hypercalcemia, renal insufficiency, anemia, and infections. The NCCN Multiple Myeloma Panel members have developed guidelines for the management of patients with various plasma cell dyscrasias, including solitary plasmacytoma, smoldering myeloma, multiple myeloma, systemic light chain amyloidosis, and Waldenström's macroglobulinemia...
February 2017: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/28181278/hematology-patient-reported-symptom-screen-to-assess-quality-of-life-for-al-amyloidosis
#20
Rahma Warsame, Shaji K Kumar, Morie A Gertz, Martha Q Lacy, Francis K Buadi, Suzanne R Hayman, Nelson Leung, David Dingli, John A Lust, Yi Lin, Stephen Russell, Prashant Kapoor, Ronald S Go, Taxiarchis Kourelis, Wilson Gonsalves, Steven R Zeldenrust, Robert A Kyle, S Vincent Rajkumar, Tyler Zemla, Jeffrey Sloan, Angela Dispenzieri
Patients with light chain amyloidosis (AL) often have delayed diagnosis and present with significant symptomatology; this may result in decreased quality of life (QOL). We prospectively employ a "Hematology Patient Reported Symptom Screen" (HPRSS) which is three questions about fatigue, pain and QOL, scored 0-10. The aim of this study is to better understand QOL and determine if HPRSS parameters predict for clinical outcomes. From 2009-2014, 302 newly diagnosed AL patients were included. Baseline median scores [interquartile range] for fatigue, pain, and QOL were 6 [3,7], 2 [0,5], 5 [3,8], respectively...
February 9, 2017: American Journal of Hematology
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