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AL amyloidosis treatment

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https://www.readbyqxmd.com/read/29660165/usefulness-of-gastroduodenal-biopsy-in-the-differential-diagnosis-of-systemic-ah-amyloidosis-from-systemic-al-amyloidosis
#1
Shojiro Ichimata, Mikiko Kobayashi, Hisashi Shimojo, Nagaaki Katoh, Masahide Yazaki, Hiroyuki Kanno
AIMS: Immunoglobulin (Ig) heavy chain amyloidosis (AH amyloidosis) is Ig-related amyloidosis classified together with Ig light chain amyloidosis (AL amyloidosis). Compared with AL amyloidosis, patients with AH amyloidosis exhibit a better prognosis and they may not need an aggressive treatment. Thus, the accurate diagnosis is essential for management of Ig-related amyloidosis patients. For definite diagnosis of AH amyloidosis, biochemical analyses are usually needed. However, these analyses can be performed in limited facilities...
April 16, 2018: Histopathology
https://www.readbyqxmd.com/read/29651545/extracardiac-18-f-florbetapir-imaging-in-patients-with-systemic-amyloidosis-more-than-hearts-and-minds
#2
T Wagner, J Page, M Burniston, A Skillen, J C Ross, R Manwani, D McCool, P N Hawkins, Ashutosh D Wechalekar
PURPOSE: 18 F-Florbetapir has been reported to show cardiac uptake in patients with systemic light-chain amyloidosis (AL). This study systematically assessed uptake of 18 F-florbetapir in patients with proven systemic amyloidosis at sites outside the heart. METHODS: Seventeen patients with proven cardiac amyloidosis underwent 18 F-florbetapir PET/CT imaging, 15 with AL and 2 with transthyretin amyloidosis (ATTR). Three patients had repeat scans. All patients had protocolized assessment at the UK National Amyloidosis Centre including imaging with 123 I-serum amyloid P component (SAP)...
April 12, 2018: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/29627865/advances-in-treatment-of-cardiac-amyloid
#3
REVIEW
Cherie N Dahm, R Frank Cornell, Daniel J Lenihan
Systemic amyloidosis represents a complex group of diseases with a common feature characterized by misfolded autologous proteins depositing into tissues or organs throughout the body. Light chain amyloidosis (AL) and transthyretin (TTR) amyloid are the two most prevalent forms of this disease that commonly results in cardiac amyloidosis. In both of these conditions, the myocardium is a frequent site of infiltration and end-organ involvement often with devastating consequences. With cardiac amyloidosis becoming an increasingly identified disease that has previously been under-recognized, the purpose of this comprehensive review is to focus on the diagnosis and treatment of these two types of cardiac amyloidosis including a contemporary update on currently available therapies being investigated in clinical trials...
April 7, 2018: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29627464/letter-to-the-editor
#4
Reza Khodarahmi
I read with great interest the paper by Dehvari and Ghahghaei (Dehvari and Ghahghaei, 2018 [1]). Their paper aimed to prove that biosynthesized AgNPs mediated by Pulicaria undulata L. has the capability in inhibiting amyloid fibril formation and thus could be considered as a therapeutic agent in the treatment of amyloidosis disorders. According to the literature (Jangholi et al., 2018 [2]), Thioflavin T (ThT) is a commonly used probe to monitor in vitro amyloid fibril formation. Also, since ThT fluorescence originates only from the bound population of the dye molecules, the several orders of magnitude increase in the fluorescence intensity makes ThT an unusually sensitive and efficient reporter...
April 5, 2018: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/29622862/the-effect-and-safety-of-bortezomib-in-the-treatment-of-al-amyloidosis-a-systematic-review-and-meta-analysis
#5
Fengjuan Jiang, Jin Chen, Hui Liu, Lijuan Li, Wenli Lu, Rong Fu
Bortezomib began to be used in the treatment of light chain (AL) amyloidosis in recent years. We performed the first meta-analysis of randomized clinical trials and clinical controlled trials to evaluate the effect and safety of bortezomib treatment for AL amyloidosis. We conducted a search (until July 2016) in electronic databases (PubMed databases and the Cochrane Central Register of Controlled Trials bases from the year 2003). There were 205 records we searched and eight studies was included (n = 617 persons)...
April 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29618684/-successful-treatment-with-thalidomide-combined-therapy-in-an-amyloidosis-complicated-multiple-myeloma-patient-refractory-to-bortezomib-lenalidomide-and-pomalidomide
#6
Yuka Aoki, Toshiaki Hayashi, Hiroshi Ikeda, Tadao Ishida
A 77-year-old man suffering from back and arm pain was referred for anemia to the hospital by an orthopedic clinic. Serum examination of the patient revealed monoclonal IgA, and he consulted the Sapporo Medical University Hospital, where he was diagnosed with multiple myeloma complicated with AL amyloidosis. He was then enrolled for a randomized double-blind study aimed to compare between melphalan-prednisone (MP) and thalidomide-melphalan-prednisone (MPT) treatments, which revealed the patient to be in the MP arm...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29577600/quantification-of-number-of-cd38-sites-on-bone-marrow-plasma-cells-in-patients-with-light-chain-amyloidosis-and-smoldering-multiple-myeloma
#7
Katharina Kriegsmann, Tobias Dittrich, Brigitte Neuber, Mohamed H S Awwad, Ute Hegenbart, Hartmut Goldschmidt, Jens Hillengass, Dirk Hose, Anja Seckinger, Carsten Müller-Tidow, Anthony D Ho, Stefan Schönland, Michael Hundemer
BACKGROUND: Recent approaches in multiple myeloma (MM) treatment have targeted CD38. As antigen expression levels on plasma cells (PCs) were demonstrated to affect response to monoclonal antibody (mAb) treatment, a precise characterization of PC phenotype is warranted. METHODS: Anti-CD38 mAb (isatuximab) was tested for antibody-dependent cellular cytotoxicity (ADCC) in MM cell lines. Quantification of the number of sites (NOS) of CD38 on bone marrow PCs and other immune cells obtained from light chain (AL) amyloidosis (n=46) and smoldering multiple myeloma (SMM) patients (n=19) was performed with two different quantitative flow cytometry (QFCM) applications...
March 25, 2018: Cytometry. Part B, Clinical Cytometry
https://www.readbyqxmd.com/read/29559907/use-of-eflornithine-dfmo-in-the-treatment-of-early-alzheimer-s-disease-a-compassionate-use-single-case-study
#8
Jessica Alber, Kelly McGarry, Richard B Noto, Peter J Snyder
Background: Recent genome-wide association screening (GWAS) studies have linked Alzheimer's disease (AD) neuropathology to gene networks that regulate immune function. Kan et al. recently reported that Arg1 (an anti-inflammatory gene that codes for arginase-1) is expressed in parts of the brain associated with amyloidosis prior to the onset of neuronal loss, suggesting that chronic brain arginine deprivation promotes AD-related neuropathology. They blocked arginine catabolism in their mouse AD model by administration of eflornithine (DFMO) to juvenile animals, effectively blocking the expression of AD-related amyloid pathology as the mice aged...
2018: Frontiers in Aging Neuroscience
https://www.readbyqxmd.com/read/29558277/stem-cell-transplantation-for-light-chain-amyloidosis-decreased-early-mortality-over-time
#9
M Hasib Sidiqi, Mohammed A Aljama, Francis K Buadi, Rahma M Warsame, Martha Q Lacy, Angela Dispenzieri, David Dingli, Wilson I Gonsalves, Shaji Kumar, Prashant Kapoor, Taxiarchis Kourelis, William J Hogan, Morie A Gertz
Purpose Autologous stem-cell transplantation (ASCT) has been used in patients with immunoglobulin light chain (AL) amyloidosis for more than two decades. Early experience raised concerns regarding safety with high early-mortality rates. Patients and Methods We report 20 years of experience with ASCT for AL amyloidosis at the Mayo Clinic Rochester. In all, 672 consecutive patients receiving ASCT for AL amyloidosis were divided into three cohorts on the basis of date of transplantation (cohort 1, 1996-2002 [n = 124]; cohort 2, 2003-2009 [n = 302]; and cohort 3, 2010-2016 [n = 246])...
March 20, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/29531659/light-chain-amyloidosis
#10
REVIEW
Paolo Milani, Giampaolo Merlini, Giovanni Palladini
Light chain (AL) amyloidosis is caused by a usually small plasma-cell clone that is able to produce the amyloidogenic light chains. They are able to misfold and aggregate, deposit in tissues in the form of amyloid fibrils and lead to irreversible organ dysfunction and eventually death if treatment is late or ineffective. Cardiac damage is the most important prognostic determinant. The risk of dialysis is predicted by the severity of renal involvement, defined by the baseline proteinuria and glomerular filtration rate, and by the response to therapy...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29529877/digoxin-use-in-systemic-light-chain-al-amyloidosis-contra-indicated-or-cautious-use
#11
Eli Muchtar, Morie A Gertz, Shaji K Kumar, Grace Lin, Barry Boilson, Alfredo Clavell, Martha Q Lacy, Francis K Buadi, Suzanne R Hayman, Prashant Kapoor, David Dingli, S Vincent Rajkumar, Angela Dispenzieri, Martha Grogan
AIM: Digoxin is considered contraindicated in light-chain (AL) amyloidosis, given reports of increased toxicity published 30-50 years ago. We sought to determine the frequency of digoxin toxicity in patients with AL. METHODS: We identified 107 patients with AL amyloidosis who received digoxin between 2000 and 2015. RESULTS: The median age was 65 and the median digoxin dose and estimated glomerular filtration rate were 0.125 mg/d and 55 ml/min/1...
March 12, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29506729/common-presentation-of-rare-diseases-left-ventricular-hypertrophy-and-diastolic-dysfunction
#12
Ales Linhart, Franco Cecchi
Left ventricular hypertrophy may be a consequence of a hemodynamic overload or a manifestation of several diseases affecting different structural and functional proteins of cardiomyocytes. Among these, sarcomeric hypertrophic cardiomyopathy (HCM) represents the most frequent cause. In addition, several metabolic diseases lead to myocardial thickening, either due to intracellular storage (glycogen storage and lysosomal diseases), extracellular deposition (TTR and AL amyloidosis) or due to abnormal energy metabolism (mitochondrial diseases)...
April 15, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29483709/prognostic-significance-of-circulating-plasma-cells-by-multi-parametric-flow-cytometry-in-light-chain-amyloidosis
#13
Surbhi Sidana, Nidhi Tandon, Angela Dispenzieri, Morie A Gertz, David Dingli, Dragan Jevremovic, William G Morice, Prashant Kapoor, Taxiarchis V Kourelis, Martha Q Lacy, Suzanne R Hayman, Francis K Buadi, Nelson Leung, Ronald S Go, Yi Lin, Stephen J Russell, John A Lust, Steven R Zeldenrust, Rahma Warsame, Yi L Hwa, Miriam Hobbs, Amie Fonder, Robert A Kyle, S Vincent Rajkumar, Shaji K Kumar, Wilson I Gonsalves
We evaluated the prognostic impact of clonal circulating plasma cells (cPCs) detected by six-color multi-parametric flow cytometry (MFC) in light chain (AL) amyloidosis at diagnosis. Of the 154 patients who underwent MFC, cPCs were detected in 42% (n = 65) patients. Median number of cPCs was 81 per 150,000 events (range: 6-17,844). High bone marrow plasma cell percentage was an independent predictor of presence of cPCs. Presence of cPCs at diagnosis was associated with inferior overall survival (OS) (90 vs...
February 21, 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/29424556/monoclonal-gammopathy-of-undetermined-significance-in-systemic-transthyretin-amyloidosis-attr
#14
Pooja Phull, Vaishali Sanchorawala, Lawreen H Connors, Gheorghe Doros, Frederick L Ruberg, John L Berk, Shayna Sarosiek
OBJECTIVE: To identify the prevalence of monoclonal gammopathy of undetermined significance (MGUS) in patients with transthyretin (ATTR) amyloidosis. PATIENTS AND METHODS: We performed a retrospective analysis of patients with biopsy-proven ATTRwt (wild-type transthyretin amyloid protein) and genopositive ATTR V122I (valine-to-isoleucine substitution at position 122 of the TTR gene) amyloidosis evaluated at the Amyloidosis Center at Boston University and Boston Medical Center between 1 January 2003 and 31 December 2016...
February 9, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29390860/healthcare-resource-utilization-and-costs-in-amyloid-light-chain-amyloidosis-a-real-world-study-using-us-claims-data
#15
Tiffany P Quock, Tingjian Yan, Eunice Chang, Spencer Guthrie, Michael S Broder
AIM: To estimate healthcare utilization and costs in amyloid light-chain (AL) amyloidosis. PATIENTS & METHODS: AL amyloidosis patients were identified in 2007-2015 claims databases if they had ≥1 inpatient/≥2 outpatient claims consistent with AL amyloidosis and received ≥1 AL-specific treatment. Descriptive statistics were reported. RESULTS: 50.1% (n = 3670) were admitted ≥1 time during the year, 11.3% (n = 827) ≥3 times. From 2007 to 2015, bortezomib use increased from 4...
February 2, 2018: Journal of Comparative Effectiveness Research
https://www.readbyqxmd.com/read/29390268/case-report-one-case-of-primary-al-amyloidosis-repeatedly-misdiagnosed-as-scleroderma
#16
Linge Sun, Lei Zhang, Wenlu Hu, Tian-Fang Li, Shengyun Liu
INTRODUCTION: Amyloid light chain (AL) results from the deposition of immunoglobulin light chain fragments, and can affect multiple organs/systems. Our patient was diagnosed as scleroderma repeatedly because of extensive skin thickening and hardening, but the treatment was not effective. We did extensive laboratory examinations including serum/urine protein electrophoresis and flow cytometry assay of bone marrow aspiration. CONCLUSION: A diagnosis of primary AL amyloidosis was established...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29359290/-renal-involvement-in-amyloidosis-and-sarcoidosis
#17
Jörg Beimler, Martin Zeier
Amyloidosis is a rare disease characterized by extracellular deposition of fibrils. Among the most common forms of systemic amyloidosis with renal involvement are AL-amyloidosis based on plasma cell dyscrasia and AA-amyloidosis in chronic inflammatory diseases. Depending on the affected renal compartment, the clinical appearance of renal amyloidosis varies. The pattern of renal amyloid deposition can be glomerular, interstitial, tubular or even vascular. Renal amyloid deposits are detected by renal biopsy. Patients with glomerular deposits typically show severe nephrotic syndrome with volume overload...
January 2018: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/29359203/rare-undiagnosed-primary-amyloidosis-unmasked-during-surgical-treatment-of-primary-hyperparathyroidism-a-case-report
#18
Kathleen C Gallagher, Ariana B Geromes, John Stokes, India A Reddy, James S Lewis, Naira Baregamian
Primary amyloidosis (PA) is a protein deposition disorder that presents with localized or multisystemic disease. The incidence is low in the general public, ranging from three to eight cases per million, and with nonspecific presenting symptoms typically occurring later in life. Due to late presentation, substantial and irreversible damage has usually already occurred by the time of the diagnosis. However, if inadvertent diagnosis occurs before irreversible damage has taken place, as it did in the following case, some patients may benefit from the disease-arresting treatment...
February 1, 2018: Journal of the Endocrine Society
https://www.readbyqxmd.com/read/29303358/treatment-patterns-and-health-care-resource-utilization-among-patients-with-relapsed-refractory-systemic-light-chain-amyloidosis
#19
Parameswaran Hari, Huamao Mark Lin, Carl V Asche, Jinma Ren, Candice Yong, Katarina Luptakova, Douglas V Faller, Vaishali Sanchorawala
BACKGROUND: Treatment for patients with systemic light chain (AL) amyloidosis remains challenging. Our study aims to describe treatment patterns for both newly diagnosed and relapsed/refractory AL (RRAL) amyloidosis, and to assess clinical outcomes, healthcare costs, and resource utilization during the first year following a diagnosis of RRAL amyloidsis. METHODS: This was a retrospective observational study of adult patients with AL amyloidosis using the US Optum administrative claims data during 1/1/2008 to 6/30/2015...
March 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29303069/unfolding-cardiac-amyloidosis-from-pathophysiology-to-cure
#20
Klemens Ablasser, Nicolas Verheyen, Theresa Glantschnig, Giulio Agnetti, Peter P Rainer
Deposition of amyloidogenic proteins leading to the formation of amyloid fibrils in the myocardium cause cardiac amyloidosis. Although any form of systemic amyloidosis can affect the heart, light-chain (AL) or transthyretin amyloidosis (ATTR) account for the majority of diagnosed cardiac amyloid deposition. The extent of cardiac disease independently predicts mortality. The reversal or arrest of adverse cardiac remodeling is the target of current therapies, as cardiac-related mortality worsens prognosis in patients where the underlying systemic amyloidosis was successfully treated...
January 4, 2018: Current Medicinal Chemistry
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