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Light chain amyloidosis

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https://www.readbyqxmd.com/read/29791070/role-of-oral-examination-in-newly-diagnosed-multiple-myeloma-patients-a-safe-and-simple-way-to-detect-light-chain-amyloidosis
#1
Merav Leiba, Suha Jarjoura, Waseem Abboud, Arnon Nagler, Ran Yahalom, Adrian Duek, Noam Yarom
OBJECTIVE: Up to 30% of multiple myeloma (MM) patients have subclinical amyloid deposits. These patients are under-recognized and are more susceptible to drug toxicity, bleeding and death. Early diagnosis and adjustment of treatment are crucial. Biopsies of oral mucosa might be a potentially useful diagnostic tool. The objective of the present study was to assess the prevalence and characteristics at presentation of oral amyloidosis in a large cohort of MM patients. METHODS: The prevalence and characteristics of oral amyloidosis in a large cohort of MM patients who were referred for oral evaluation before and during bisphosphonate therapy were assessed, retrospectively...
May 23, 2018: Oral Diseases
https://www.readbyqxmd.com/read/29782249/-transthyretin-amyloidosis-in-a-cohort-of-old-and-very-old-patients-with-chronic-heart-failure
#2
A A Poliakova, E N Semernin, M Y Sitnikova, K L Аvagyan, R V Grozov, S A Pyko, A N Krutikov, V G Davydova, K A Khmelnitskaya, M M Shavloskii, D E Korzhevskii, A Y Gudkova
BACKGROUND: Life-time diagnostics of wild type transthyretin amyloidosis (ATTR(wt)-amyloidosis) is virtually absent, even though ATTR(wt)-amyloidosis is an underestimated cause for morbidity and mortality, particularly in the older age group. AIM: To study incidence, demographic characteristics, and morpho-functional features of ATTR(wt)-amyloidosis in patients with FC IV CHF and LV hypertrophy > 15 mm according to autopsy data. MATERIALS AND METHODS: Postmortem reports were retrospectively analyzed for patients (n=141; 19 % males, 81 % females) of cardiology departments aged ≥69 with the underlying CHF syndrome...
2018: Kardiologiia
https://www.readbyqxmd.com/read/29770800/current-and-future-circulating-biomarkers-for-cardiac-amyloidosis
#3
REVIEW
Marco Luciani, Luca Troncone, Federica Del Monte
Cardiac amyloidosis (CA) comprises a heterogeneous group of medical conditions affecting the myocardium. It presents with proteinaceous infiltration with variable degrees of severity, prevalence and evolution. Despite this heterogeneity, erroneous protein folding is the common pathophysiologic process, yielding the formation of a single misfolded protein (monomer) that progressively evolves and ultimately forms amyloid fibers. Additionally, by seeding out from the organs of origin, intermediates called oligomers metastasize and restart the process...
May 17, 2018: Acta Pharmacologica Sinica
https://www.readbyqxmd.com/read/29767409/the-use-of-serum-free-light-chain-dimerization-patterns-assist-in-the-diagnosis-of-al-amyloidosis
#4
Moshe E Gatt, Batia Kaplan, Dean Yogev, Elana Slyusarevsky, Galina Pogrebijski, Sizilia Golderman, Olga Kukuy, Avi Livneh
The discrimination between benign and malignant forms of plasma cell dyscrasia (PCD) is often difficult. Free light chain monomer-dimer pattern analysis (FLC-MDPA) may assist in solving this dilemma and distinguish between AL amyloidosis and benign PCD. Serum samples of patients with AL amyloidosis and benign PCD were analysed in a blinded manner. Quantitative Western blotting was performed to estimate dimerization and clonality indices, and thereby determine the source of the tested samples, as derived either from benign or malignant PCD...
May 16, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29753692/treatment-with-bortezomib-based-therapy-followed-by-autologous-stem-cell-transplantation-improves-outcomes-in-light-chain-amyloidosis-a-retrospective-study
#5
Tania Jain, Heidi E Kosiorek, Shu T Kung, Vishal S Shah, Amylou C Dueck, Veronica Gonzalez-Calle, Susan Luft, Craig B Reeder, Roberta Adams, Pierre Noel, Jeremy T Larsen, Joseph Mikhael, Leif Bergsagel, A Keith Stewart, Rafael Fonseca
BACKGROUND: The hematologic response is critical in patients with light chain amyloidosis because a good response is known to improve organ response and overall survival. We present a retrospective analysis to compare the hematologic and organ response in patients who received bortezomib-based therapy before autologous stem cell transplantation (ASCT) versus those who received non-bortezomib-based therapy before ASCT and those who underwent ASCT at diagnosis. PATIENTS AND METHODS: Of a total of 63 patients who underwent ASCT for light chain amyloidosis, 34 received bortezomib-based therapy before ASCT (Bor-ASCT) and 29 did not receive bortezomib therapy (non-Bor-ASCT)...
May 4, 2018: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/29752394/echocardiography-and-survival-in-light-chain-cardiac-amyloidosis-back-to-basics
#6
EDITORIAL
Omar K Siddiqi, Vaishali Sanchorawala, Frederick L Ruberg
No abstract text is available yet for this article.
May 2018: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29752392/independent-prognostic-value-of-stroke-volume-index-in-patients-with-immunoglobulin-light-chain-amyloidosis
#7
(no author information available yet)
BACKGROUND: Heart involvement is the most important prognostic determinant in AL amyloidosis patients. Echocardiography is a cornerstone for the diagnosis and provides important prognostic information. METHODS AND RESULTS: We studied 754 patients with AL amyloidosis who underwent echocardiographic assessment at the Mayo Clinic, including a Doppler-derived measurement of stroke volume (SV) within 30 days of their diagnosis to explore the prognostic role of echocardiographic variables in the context of a well-established soluble cardiac biomarker staging system...
May 2018: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29748430/epidemiology-of-al-amyloidosis-a-real-world-study-using-us-claims-data
#8
Tiffany P Quock, Tingjian Yan, Eunice Chang, Spencer Guthrie, Michael S Broder
Amyloid light-chain (AL) amyloidosis is a rare disease caused by extracellular deposition of misfolded immunoglobulin light chains. This study aimed to provide an up-to-date estimate of prevalence and incidence of AL amyloidosis in the United States. Using claims databases from years 2007 to 2015, adults ≥18 years old with AL amyloidosis were included if they had (1) at least 1 inpatient or 2 outpatient claims consistent with AL amyloidosis and (2) received 1 AL-specific treatment. Prevalence was calculated as the number of AL patients divided by the number of enrollees on June 30th of each calendar year...
May 22, 2018: Blood Advances
https://www.readbyqxmd.com/read/29733684/mri-feature-tracking-strain-is-prognostic-for-all-cause-mortality-in-al-amyloidosis
#9
Jeffery E Illman, Shivaram P Arunachalam, Arvin Arani, Ian Cheng-Yi Chang, James F Glockner, Angela Dispenzieri, Martha Grogan, Philip A Araoz
OBJECTIVES: Cardiac involvement is a major determinate of mortality in light chain (AL) amyloidosis. Cardiac magnetic resonance imaging (MRI) feature tracking (FT) strain is a new method for measuring myocardial strain. This study retrospectively evaluated the association of MRI FT strain with all-cause mortality in AL amyloidosis. MATERIALS AND METHODS: Seventy-six patients with newly diagnosed AL amyloidosis underwent cardiac MRI. 75 had images suitable for MRI FT strain analysis...
May 7, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29709420/outcome-of-patients-with-cardiac-amyloidosis-admitted-to-an-intensive-care-unit-for-acute-heart-failure
#10
Thomas d'Humières, Damien Fard, Thibaud Damy, Francois Roubille, Arnaud Galat, Huy-Long Doan, Leopold Oliver, Jean-Luc Dubois-Randé, Pierre Squara, Pascal Lim, Julien Ternacle
BACKGROUND: The outcome of cardiac amyloidosis (CA) has been reported mainly in stable populations; limited data are available in patients referred for acute heart failure (AHF) to an intensive cardiac care unit (ICCU). AIMS: To address the characteristics and outcomes of patients with confirmed CA admitted to an ICCU for AHF and then to identify the predictors of evolution to cardiogenic shock. METHODS: All patients with CA referred to an ICCU for AHF between 2009 and 2015 were included...
April 27, 2018: Archives of Cardiovascular Diseases
https://www.readbyqxmd.com/read/29707360/wild-type-transthyretin-cardiac-amyloidosis-attrwt-ca-previously-known-as-senile-cardiac-amyloidosis-clinical-presentation-diagnosis-management-and-emerging-therapies
#11
REVIEW
Ilia G Halatchev, Jingsheng Zheng, Jiafu Ou
Cardiac amyloidosis is thought to be a rare group of diseases caused by extracellular deposition of misfolded proteins in the extracellular cardiac matrix resulting in heart failure with preserved ejection fraction (HFpEF). This review focuses on the similarities and differences between the pathophysiology, clinical presentation and diagnostic tests of wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) compared to immunoglobulin light chain amyloidosis and hereditary cardiac amyloidosis. We address some obstacles to timely diagnosis and opportunities for management of the clinical symptoms as well as possibility of future novel disease modifying therapies...
March 2018: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29703839/the-complexity-and-heterogeneity-of-monoclonal-immunoglobulin-associated-renal-diseases
#12
Sanjeev Sethi, S Vincent Rajkumar, Vivette D D'Agati
Monoclonal gammopathies are characterized by the overproduction of monoclonal Ig (MIg) detectable in the serum or urine resulting from a clonal proliferation of plasma cells or B lymphocytes. The underlying hematologic conditions range from malignant neoplasms of plasma cells or B lymphocytes, including multiple myeloma and B-cell lymphoproliferative disorders, to nonmalignant small clonal proliferations. The term MGUS implies presence of an MIg in the setting of a "benign" hematologic condition without renal or other end organ damage...
April 27, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29700090/cardiac-amyloidosis
#13
Ana Martinez-Naharro, Philip N Hawkins, Marianna Fontana
Systemic amyloidosis comprises an uncommon group of disorders caused by the extracellular deposition of misfolded proteins in various organs. Cardiac amyloid deposition, causing an infiltrative/restrictive cardiomyopathy, is a frequent feature of amyloidosis and a major determinant of survival. It may be the presenting feature of the disease or may be identified while investigating a patient presenting with other organ involvement. The need for a high index of suspicion and the critical importance of precise biochemical typing of the amyloid deposits is paramount in light of recent therapeutic advances that can significantly improve prognosis...
April 1, 2018: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/29697673/-solitary-epithelioid-hemangioendothelioma-concurrent-with-nodular-parenchymal-amyloidosis-of-the-lung-and-rosai-dorfman-disease
#14
O V Dolzhansky, E M Paltseva, M M Morozova, D V Bazarov, E V Boranov, D N Fedorov
The paper describes a case of solitary epithelioid hemangioendothelioma concurrent with nodular parenchymal AL amyloidosis of the lung and Rosai-Dorfman disease in a 70-year-old woman. The core of the tumor was represented by bone tissue with dendriform ossification, as well as by amyloid that showed green apple birefringence at polarized light microscopy. The peripheral portions of the tumor and the myxohyaline stroma exhibited slit-like structures, epithelioid and fusiform cells with small cytoplasmic vacuoles...
2018: Arkhiv Patologii
https://www.readbyqxmd.com/read/29696194/rationale-and-design-of-dual-study-doxycycline-to-upgrade-response-in-light-chain-al-amyloidosis-dual-a-phase-2-pilot-study-of-a-two-pronged-approach-of-prolonged-doxycycline-with-plasma-cell-directed-therapy-in-the-treatment-of-al-amyloidosis
#15
Anita D'Souza, Kathryn Flynn, Saurabh Chhabra, Binod Dhakal, Mehdi Hamadani, Kirsten Jacobsen, Marcelo Pasquini, Dorothee Weihrauch, Parameswaran Hari
Light chain (AL) amyloidosis is a plasma cell neoplasm associated with insoluble fibril deposition from clonal immunoglobulin chains systemically. The disease is associated with high early mortality and morbidity owing to advanced organ deposition as well as lack of proven de-fibrillogenic therapies. Pre-clinical and retrospective clinical data suggests that doxycycline has benefit in AL amyloidosis. The ongoing DUAL study is a single center, open label, phase 2 study in which patients with AL amyloidosis who are undergoing clone-directed therapy for the underlying neoplasm with oral doxycycline given for 1 year to test the hypothesis that prolonged doxycycline use will be safe, feasible, and lead to reduced early mortality in systemic AL amyloidosis and hasten organ amyloid response...
December 2017: Contemporary Clinical Trials Communications
https://www.readbyqxmd.com/read/29680336/cardiac-structural-and-functional-consequences-of-amyloid-deposition-by-cardiac-magnetic-resonance-and-echocardiography-and-their-prognostic-roles
#16
Daniel S Knight, Giulia Zumbo, William Barcella, Jennifer A Steeden, Vivek Muthurangu, Ana Martinez-Naharro, Thomas A Treibel, Amna Abdel-Gadir, Heerajnarain Bulluck, Tushar Kotecha, Rohin Francis, Tamer Rezk, Candida C Quarta, Carol J Whelan, Helen J Lachmann, Ashutosh D Wechalekar, Julian D Gillmore, James C Moon, Philip N Hawkins, Marianna Fontana
OBJECTIVES: This cross-sectional study aimed to describe the functional and structural cardiac abnormalities that occur across a spectrum of cardiac amyloidosis burden and to identify the strongest cardiac functional and structural prognostic predictors in amyloidosis using cardiac magnetic resonance (CMR) and echocardiography. BACKGROUND: Cardiac involvement in light chain and transthyretin amyloidosis is the main driver of prognosis and influences treatment strategies...
April 13, 2018: JACC. Cardiovascular Imaging
https://www.readbyqxmd.com/read/29674497/plasma-cell-proliferative-index-predicts-outcome-in-immunoglobulin-light-chain-amyloidosis-treated-with-stem-cell-transplantation
#17
M Hasib Sidiqi, Mohammed Aljama, Dragan Jevremovic, William G Morice, Michael Timm, Francis K Buadi, Rahma Warsame, Martha Q Lacy, Angela Dispenzieri, David Dingli, Wilson I Gonsalves, Shaji Kumar, Prashant Kapoor, Taxiarchis Kourelis, Nelson Leung, William J Hogan, Morie Gertz
The plasma cell proliferative index provides an insight into plasma cell biology in plasma cell disorders and is an important prognostic marker in myeloma and smoldering myeloma. We analyzed the prognostic impact of the plasma cell proliferative index in 513 patients with systemic AL Amyloidosis undergoing stem cell transplantation at the Mayo Clinic between January 2003 and 31 August 2016. Two cohorts were identified; Low or Elevated plasma cell proliferative index. Patients with an Elevated plasma cell proliferative index had more cardiac involvement (56% vs 44%; p=0...
April 19, 2018: Haematologica
https://www.readbyqxmd.com/read/29668344/phenotypic-plasticity-of-mesenchymal-stem-cells-is-crucial-for-mesangial-repair-in-a-model-of-immunoglobulin-light-chain-associated-mesangial-damage
#18
Guillermo A Herrera, Jiamin Teng, Chun Zeng, Hongzhi Xu, Man Liang, J Steven Alexander, Bing Liu, Chris Boyer, Elba A Turbat-Herrera
Mesangiopathies produced by glomerulopathic monoclonal immunoglobulin light chains (GLCs) acting on the glomerular mesangium produce two characteristic lesions: AL-amyloidosis (AL-Am) and light chain deposition disease (LCDD). In both cases, the pathology is centered in the mesangium, where initial and progressive damage occurs. In AL-Am the mesangial matrix is destroyed and replaced by amyloid fibrils and in LCDD, the mesangial matrix is increased and remodeled. The collagen IV rich matrix is replaced by tenascin...
April 18, 2018: Ultrastructural Pathology
https://www.readbyqxmd.com/read/29664336/left-ventricular-myocardial-deformation-on-cine-mr-images-relationship-to-severity-of-disease-and-prognosis-in-amyloid-light-chain-amyloidosis
#19
Ke Wan, Jiayu Sun, Dan Yang, Hong Liu, Jie Wang, Wei Cheng, Qing Zhang, Zhi Zeng, Tianjing Zhang, Andreas Greiser, Marie-Pierre Jolly, Yuchi Han, Yucheng Chen
Purpose To measure left ventricular (LV) myocardial strain with cine magnetic resonance (MR) imaging and a deformable registration algorithm (DRA) and to assess the prognostic value of myocardial strain in patients with light-chain (AL) amyloidosis. Materials and Methods In this prospective study, 78 consecutive patients with AL amyloidosis who underwent contrast material-enhanced cardiac MR imaging were enrolled at West China Hospital. LV myocardial strains and late gadolinium enhancement (LGE) were evaluated...
April 17, 2018: Radiology
https://www.readbyqxmd.com/read/29660165/usefulness-of-gastroduodenal-biopsy-in-the-differential-diagnosis-of-systemic-ah-amyloidosis-from-systemic-al-amyloidosis
#20
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
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