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Al Amyloidosis

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https://www.readbyqxmd.com/read/29791180/-heart-transplantation-and-follow-up-treatment-with-al-amyloidosis-in-5-patients
#1
Zdeněk Adam, Eva Ozábalová, Petr Němec, Helena Bedáňová, Milan Kuman, Jan Krejčí, Lenka Špinarová, Víta Žampachová, Zdeňka Čermáková, Luděk Pour, Marta Krejčí, Viera Sanecká, Martin Štork, Tomáš Pika, Jan Straub, Dagmar Adamová, Yvetta Stavařová, Zdeněk Král, Jiří Mayer
The prognosis for patients with cardiac impairment due to AL-amyloid deposition and severe cardiac insufficiency is poor, with a survival median in the order of months. The classical treatment of AL-amyloidosis in combination with cardiac insufficiency is very poorly tolerated and the treatment of such patients is associated with considerably higher mortality than among other patients with AL-amyloidosis. If, however, patients with an isolated or another dominating cardiac impairment, without severe damage to other organs and tissues, have a heart transplant performed, their cardiovascular condition will significantly improve as a result, along with their ability to tolerate any kind of treatment for AL-amyloidosis including that using high-dose chemotherapy with a transplant of autologous hematopoietic stem cells...
2018: Vnitr̆ní Lékar̆ství
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
#2
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/29786186/-ebv-associated-pneumonia-in-patient-with-granulomatosis-with-polyangiitis-gpa-in-immunosoppressive-therapy-treated-with-aciclovir
#3
Nicola Mongera, Vittorio Di Maso, Elisabetta Ermacora, Michele Carraro, Cristina Bregant, Martina Pian, Umberto Savi, Eric Lorenzon, Giuliano Boscutti
We describe the case of a 74-year-old man admitted to our Nephrology Unit with nephrotic syndrome and mild kidney disease. A complete panel of laboratoristic and instrumental tests did not provide useful information for diagnosis. No specific signs or symptoms suggested the presence of AL amyloidosis. As a matter of fact, diagnosis was reached thanks to the hystopathologic examination of renal tissue and bone marrow, since the associated B-cell lymphoproliferative disorder had not revealed itself through serum and urine electrophoresis and immunofixation...
May 2018: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/29786185/-an-unusual-presentation-of-amyloidosis-al
#4
Anna Zito, Antonio De Pascalis, Paolo Ria, Annarita Armeni, Alessandro D'Amelio, Marcello Napoli
We describe the case of a 74-year-old man admitted to our Nephrology Unit with nephrotic syndrome and mild kidney disease. A complete panel of laboratoristic and instrumental tests did not provide useful information for diagnosis. No specific signs or symptoms suggested the presence of AL amyloidosis. As a matter of fact, diagnosis was reached thanks to the hystopathologic examination of renal tissue and bone marrow, since the associated B-cell lymphoproliferative disorder had not revealed itself through serum and urine electrophoresis and immunofixation...
May 2018: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/29767409/the-use-of-serum-free-light-chain-dimerization-patterns-assist-in-the-diagnosis-of-al-amyloidosis
#5
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/29752392/independent-prognostic-value-of-stroke-volume-index-in-patients-with-immunoglobulin-light-chain-amyloidosis
#6
(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
#7
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
#8
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/29731423/evaluation-of-a-new-continuous-mononuclear-cell-collection-procedure-in-a-single-transplant-center-cohort-enriched-for-al-amyloidosis-patients
#9
Anita Pudusseri, India Smith, Diane Sarnacki, Dina Brauneis, Anthony Shelton, Vaishali Sanchorawala, J Mark Sloan, Shayna Sarosiek, Karen Quillen
BACKGROUND: The Spectra Optia continuous mononuclear cell (CMNC) program is newly available, and herein validated in a single-center cohort enriched with AL amyloidosis patients to collect a target CD34+ yield of 2.5 × 106 cells/kg within 2 days. METHODS: Consecutive autologous transplant patients in 2016 are included. Patients undergo leukapheresis with Optia CMNC and Spectra v4.7 over a 2-day cycle. Data collection includes collection efficiency, adverse events and engraftment kinetics...
April 26, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29719808/primary-endobronchial-amyloidosis-a-rare-case-of-endobronchial-tumor
#10
Amos Lal, Jamal Akhtar, Mohammad Saud Khan, Yayan Chen, Yaron Goldman
Primary localized endobronchial amyloidosis is a rare entity, as pulmonary amyloidosis most commonly occurs as a part of systemic AL amyloidosis. It can be asymptomatic or can present with nonspecific symptoms such as progressive dyspnea, cough, wheezing and rarely respiratory failure. It is frequently misdiagnosed as asthma, COPD or pneumonia. Solitary endobronchial amyloidosis having a nodular appearance can mimic endobronchial tumor. The diagnosis is usually delayed by 8-37 months. The average life expectancy for primary tracheobronchial amyloidosis is approximately 9 years, so the early diagnosis of this disease is very critical to improve the prognosis of patients...
2018: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/29709420/outcome-of-patients-with-cardiac-amyloidosis-admitted-to-an-intensive-care-unit-for-acute-heart-failure
#11
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/29706127/utility-of-the-18-f-florbetapir-positron-emission-tomography-in-systemic-amyloidosis
#12
Jaume Mestre-Torres, Carles Lorenzo-Bosquet, Gemma Cuberas-Borrós, Mercedes Gironella, Roser Solans-Laque, Andreu Fernández-Codina, Segundo Bujan-Rivas, Joan Castell-Conesa, Fernando Martínez-Valle
Amyloidosis comprises a group of heterogeneous conditions. To ascertain the burden of disease is important because it can determine the treatment as well as the evolution of the disease. Recent reports have shown good results in diagnosing cardiac amyloidosis using 18 F-florbetapir. We hypothesize that combining whole body PET/CT with 18 F-Florbetapir can be useful to characterize the burden of the disease. We included 25 patients, 13 of them with different types of amyloidosis, and 12 with Alzheimer's disease as controls...
April 28, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
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/29692592/revisiting-renal-amyloidosis-with-clinicopathological-characteristics-grading-and-scoring-a-single-institutional-experience
#16
Abhiram Kalle, Archana Gudipati, Sree Bhushan Raju, Karthik Kalidindi, Swarnalatha Guditi, Gangadhar Taduri, Megha S Uppin
INTRODUCTION: Kidney involvement is a major cause of mortality in systemic amyloidosis. Glomerulus is the most common site of deposition in renal amyloidosis, and nephrotic syndrome is the most common presentation. Distinction between AA and AL is done using immunofluorescence (IF) and immunohistochemistry (IHC). Renal biopsy helps in diagnosis and also predicting the clinical course by applying scoring and grading to the biopsy findings. MATERIALS AND METHODS: The study includes all cases of biopsy-proven renal amyloidosis from January 2008 to May 2017...
April 2018: Journal of Laboratory Physicians
https://www.readbyqxmd.com/read/29685951/dual-therapy-for-a%C3%AE-amyloidosis-in-ad-a-successful-one-two-combo
#17
Tirth K Patel, David M Holtzman
In this issue, Chiang et al. (https://doi.org/10.1084/jem.20171484) make a notable contribution to Alzheimer disease (AD) therapeutics in a thorough and rigorous study demonstrating superior efficacy of dual therapy against Aβ in a mouse model of amyloid β deposition.
April 23, 2018: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/29674497/plasma-cell-proliferative-index-predicts-outcome-in-immunoglobulin-light-chain-amyloidosis-treated-with-stem-cell-transplantation
#18
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
#19
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
#20
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
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