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Al Amyloidosis

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https://www.readbyqxmd.com/read/27908298/reply-to-long-term-tocilizumab-efficacy-in-a-patient-with-psoriatic-arthritis-and-aa-amyloidosis-dinoia-et-al
#1
Thirusha Lane, Helen J Lachmann
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October 27, 2016: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/27904139/interphase-fluorescence-in-situ-hybridization-ifish-in-untreated-al-amyloidosis-has-an-independent-prognostic-impact-by-abnormality-type-and-treatment-category
#2
E Muchtar, A Dispenzieri, S K Kumar, R P Ketterling, D Dingli, M Q Lacy, F K Buadi, S R Hayman, P Kapoor, N Leung, R Chakraborty, W Gonsalves, R Warsame, T V Kourelis, S Russell, J A Lust, Y Lin, R S Go, S Zeldenrust, R A Kyle, S V Rajkumar, M A Gertz
The significance of interphase fluorescence in-situ hybridization (iFISH) by regimen type was assessed in 692 AL amyloidosis patients with iFISH at diagnosis. First line treatment was categorized as stem cell transplant and three non-transplant regimens. The most common abnormality was t(11;14) (49% of patients) followed by monosomy 13/del(13q) (36%) and trisomies (26%). A lower rate of very good partial response (VGPR) or better was observed in patients with t(11;14) treated with bortezomib-based (52 vs 77%; P=0...
December 1, 2016: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/27900617/new-and-evolving-concepts-regarding-the-prognosis-and-treatment-of-cardiac-amyloidosis
#3
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/27885144/treatment-of-multiple-myeloma-in-a-heart-transplant-recipient-focus-on-the-immunomodulatory-effects-of-lenalidomide
#4
Lola Xie, Bartosz Jozwik, Phillip Weeks, L Maximilian Buja, Robert Brown, Sriram Nathan, Keshava Rajagopal, Christina Paruthi, Biswajit Kar, Pranav Loyalka, Indranee Rajapreyar
Malignancy following solid organ transplant remains a significant threat to the survival of cardiac transplant recipients. Plasma cell dyscrasias including multiple myeloma have been encountered in this population, and medication treatments traditionally used to treat these disorders demonstrate immunomodulatory effects that may have implications on the transplanted allograft. Lenalidomide is an immunomodulatory agent that has been used to treat plasma cell disorders, including light-chain amyloidosis (AL) and multiple myeloma, and represents such a class of medications in which the risks and benefits in the solid organ transplant population remain to be fully elucidated...
November 24, 2016: Progress in Transplantation
https://www.readbyqxmd.com/read/27879147/pre-transplantation-novel-agent-induction-predicts-progression-free-survival-for-patients-with-immunoglobulin-light-chain-amyloidosis-undergoing-high-dose-melphalan-and-autologous-stem-cell-transplantation
#5
Andrew J Cowan, Zandra K Klippel, Philip A Stevenson, Teresa S Hyun, Sherilyn Tuazon, Pamela S Becker, Damian J Green, Leona A Holmberg, David G Coffey, Ajay K Gopal, Edward N Libby
INTRODUCTION: High-dose melphalan and autologous stem cell transplantation (HDM/SCT) is an effective treatment modality for immunoglobulin light-chain (AL) amyloidosis; however, its application remains restricted to patients with good performance status and limited organ involvement. In recent years, the paradigm for AL amyloidosis has changed with the introduction of novel agents such as immunomodulatory drugs (IMiDs) and proteasome inhibitors (PIs). We hypothesized that use of novel agent induction regimens has improved outcomes for patients with AL amyloidosis undergoing HDM/SCT at our center...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27878699/differentiation-of-light-chain-cardiac-amyloidosis-from-hypertrophic-cardiomyopathy-using-myocardial-mechanical-parameters-by-velocity-vector-imaging-echocardiography
#6
Lu Zhang, Xiao Zhou, Jing Wang, Yang Mu, Bohan Liu, Wenqing Lv, Ye Wang, Hongwei Liu, Hongbin Liu, Guang Zhi
We aimed to evaluate the diagnostic efficacy of layered velocity vector imaging (VVI)-derived left ventricular (LV) mechanical parameters in the differential diagnosis of primary light-chain cardiac amyloidosis (AL-CA) and hypertrophic cardiomyopathy (HCM). We recruited 35 subjects with histologically-diagnosed AL-CA, 35 subjects with HCM, and 30 age-matched healthy controls. We used conventional echocardiography and electrocardiogram to evaluate general heart function and electrophysiology properties. Furthermore, we applied two-dimensional VVI echocardiography to assess the layered mechanical parameters during systole, including endocardial and epicardial longitudinal strain (ENDO and EPI LSsys), circumferential strain (CSsys), radial strain (RSsys), rotation (ROT) and twist (TWI), in different LV walls and levels...
November 23, 2016: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/27872470/hereditary-amyloidosis-with-recurrent-lung-infiltrates
#7
Alberto E Revelo, Crischelle Magaspi, George Maguire, Wilbert S Aronow
BACKGROUND Amyloidosis is a protein conformational disorder characterized by extracellular deposition of amyloid fibrils in extracellular tissue. Lung involvement is most commonly caused by secondary AL amyloidosis. The familial autosomal-dominant senile transthyretin (ATTR) disease manifests mainly as polyneuropathy and restrictive cardiomyopathy denoting the name familial amyloidotic polyneuropathy (FAP). Rarely, this form manifests with clinical and radiologically relevant respiratory tract symptoms and lung involvement...
November 22, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27862330/predictors-of-inferior-clinical-outcome-in-patients-with-standard-risk-multiple-myeloma
#8
Talha Badar, Samer Srour, Qaiser Bashir, Nina Shah, Gheath Al-Atrash, Chitra Hosing, Uday Popat, Yago Nieto, Robert Z Orlowski, Richard Champlin, Muzaffar H Qazilbash
INTRODUCTION: Outcome of patients with standard-risk (SR) multiple myeloma (MM) has improved; however, subsets of patients do worse than expected. We sought to identify the factors associated with inferior outcome. METHODS: We evaluated 51 patients with SR MM that received upfront autologous hematopoietic stem cell transplantation (auto-HCT) after induction and had a progression-free survival (PFS) of ≤18 months. RESULTS: The median age of patients was 61 yr...
November 9, 2016: European Journal of Haematology
https://www.readbyqxmd.com/read/27861403/localized-immunoglobulin-light-chain-amyloid-of-the-conjunctiva-confirmed-by-mass-spectrometry-without-evidence-of-systemic-disease
#9
Eric B Hamill, Preeti J Thyparampil, Michael T Yen
Current guidelines recommend a thorough systemic workup in patients with newly diagnosed immunoglobulin amyloid light chain (AL) amyloidosis because of a close association with an underlying plasma cell proliferative disorder. Rarely, however, AL amyloidosis may present as a localized, benign disease without systemic involvement. Few previous reports have documented localized AL amyloid of the conjunctiva, and all these used immunohistochemistry for amyloid typing, which can be inaccurate. The authors identified 2 cases of AL amyloidosis confirmed by mass spectrometry localized to the conjunctiva and without evidence of systemic disease as determined by thorough systemic workup...
November 15, 2016: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/27856462/clarifying-immunoglobulin-gene-usage-in-systemic-and-localized-immunoglobulin-light-chain-amyloidosis-by-mass-spectrometry
#10
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/27832515/bortezomib-dexamethasone-versus-high-dose-melphalan-for-japanese-patients-with-systemic-light-chain-al-amyloidosis-a-retrospective-single-center-study
#11
Nagaaki Katoh, Akihiro Ueno, Takuhiro Yoshida, Ko-Ichi Tazawa, Yasuhiro Shimojima, Takahisa Gono, Yoshiki Sekijima, Masayuki Matsuda, Shu-Ichi Ikeda
Bortezomib-dexamethasone (BD) and high-dose melphalan (HDM) are effective for systemic light-chain (AL) amyloidosis, but have not been compared in detail. We retrospectively investigated patients treated with BD or HDM at our center between September 2001 and June 2016. Among 234 patients, 20 were treated with BD and 30 received HDM. With the exception of age, transplant eligibility, and previous history of other chemotherapy, there were no significant differences in most background parameters between the two groups...
November 10, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27825470/monoclonal-igm-related-al-amyloidosis
#12
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/27815860/phase-2-trial-of-daily-oral-epigallocatechin-gallate-in-patients-with-light-chain-amyloidosis
#13
Sohsuke Meshitsuka, Sumito Shingaki, Masatoshi Hotta, Miku Goto, Makoto Kobayashi, Yuuichi Ukawa, Yuko M Sagesaka, Yasuyo Wada, Masanori Nojima, Kenshi Suzuki
Previous studies have suggested that an increase in mitochondrial reactive oxygen species may cause organ damage in patients with light-chain (AL) amyloidosis; however, this damage can be decreased by antioxidant-agent treatment. Epigallocatechin gallate (EGCG), the major natural catechin in green tea, has potent antioxidant activity. Because EGCG has recently been reported to have a favorable toxicity profile for treating amyloidosis, we sought to examine the clinical efficacy and toxicity of EGCG in patients with AL amyloidosis...
November 4, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27811147/development-of-the-autoinflammatory-disease-damage-index-addi
#14
Nienke M Ter Haar, Kim V Annink, Sulaiman M Al-Mayouf, Gayane Amaryan, Jordi Anton, Karyl S Barron, Susanne M Benseler, Paul A Brogan, Luca Cantarini, Marco Cattalini, Alexis-Virgil Cochino, Fabrizio De Benedetti, Fatma Dedeoglu, Adriana A De Jesus, Ornella Della Casa Alberighi, Erkan Demirkaya, Pavla Dolezalova, Karen L Durrant, Giovanna Fabio, Romina Gallizzi, Raphaela Goldbach-Mansky, Eric Hachulla, Veronique Hentgen, Troels Herlin, Michaël Hofer, Hal M Hoffman, Antonella Insalaco, Annette F Jansson, Tilmann Kallinich, Isabelle Koné-Paut, Anna Kozlova, Jasmin B Kuemmerle-Deschner, Helen J Lachmann, Ronald M Laxer, Alberto Martini, Susan Nielsen, Irina Nikishina, Amanda K Ombrello, Seza Ozen, Efimia Papadopoulou-Alataki, Pierre Quartier, Donato Rigante, Ricardo Russo, Anna Simon, Maria Trachana, Yosef Uziel, Angelo Ravelli, Marco Gattorno, Joost Frenkel
OBJECTIVES: Autoinflammatory diseases cause systemic inflammation that can result in damage to multiple organs. A validated instrument is essential to quantify damage in individual patients and to compare disease outcomes in clinical studies. Currently, there is no such tool. Our objective was to develop a common autoinflammatory disease damage index (ADDI) for familial Mediterranean fever, cryopyrin-associated periodic syndromes, tumour necrosis factor receptor-associated periodic fever syndrome and mevalonate kinase deficiency...
November 3, 2016: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/27809600/plasma-hepatocyte-growth-factor-is-a-novel-marker-of-al-cardiac-amyloidosis
#15
Kristopher J Swiger, Eitan A Friedman, Evan L Brittain, Kelsey A Tomasek, Shi Huang, Yan R Su, Douglas B Sawyer, Daniel J Lenihan
BACKGROUND: Cardiac amyloidosis is an infiltrative cardiomyopathy that is challenging to diagnose. We hypothesized that the novel biomarkers hepatocyte growth factor (HGF), galectin-3 (GAL-3), interleukin-6 (IL-6), and vascular endothelial growth factor (VEGF) would be elevated in cardiac amyloidosis and may be able to discriminate from non-cardiac systemic amyloidosis or other cardiomyopathies with similar clinical or morphologic characteristics. METHODS: Patients were selected from the Vanderbilt Main Heart Registry according to the following groups: (1) amyloid light-chain (AL) cardiac amyloidosis (n = 26); (2) transthyretin (ATTR) cardiac amyloidosis (n = 7); (3) left ventricular hypertrophy (LVH) (n = 45); (4) systolic heart failure (n = 42); and (5) non-cardiac systemic amyloidosis (n = 7)...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27804150/progressive-refractory-light-chain-amyloidosis-and-multiple-myeloma-patients-are-responsive-to-the-addition-of-clarithromycin-to-imid-based-therapy
#16
Adir Shaulov, Chezi Ganzel, Noam Benyamini, Yossef Barshay, Neta Goldschmidt, David Lavie, Diana Libster, Alex Gural, Batia Avni, Moshe E Gatt
Multiple myeloma (MM) and primary systemic light chain amyloidosis (AL) are both chronic plasma cell dyscrasias with different clinical expression but limited treatment options for relapsed refractory disease. We report the effect of the addition of clarithromycin on 31 MM and 17 AL with relapsed or refractory disease who had an insufficient response or disease progression while on an IMiD based therapy. In this high risk population, hematological response was reported in 48% of MM patients and 94% of AL patients...
November 2, 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27801739/18f-florbetaben-a-new-tool-for-amyloidosis-staging
#17
Emmanuel D'Estanque, Benjamin Chambert, Olivier Moranne, Pierre Olivier Kotzki, Vincent Boudousq
We report the case of a 73-year-old man with a documented (renal biopsy) light-chain amyloidosis (AL) imaged with F-AV-1 (F-florbetaben) compared with a volunteer. A cardiac amyloidosis was suspected. As it was an AL and not a transthyretin amyloidosis, F-FDG and F-florbetaben PET/CT were preferred to bone scan. F-FDG scintigraphy showed a focal cardiac hypermetabolism. In addition of the heart, F-florbetaben scintigraphy showed an intense spleen and thyroid pathologic uptake and a moderate salivary gland and kidney uptake...
October 31, 2016: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/27797847/amyloidosis-an-unusual-cause-of-upper-gastrointestinal-bleeding
#18
Keith Siau, Amera Elzubeir, Sheldon C Cooper, Tariq Iqbal
We report a rare case of upper gastrointestinal bleeding in a 55-year-old man with monoclonal gammopathy of unknown significance presenting with abdominal pain, weight loss and melaena. Gastroscopy was unremarkable, but melaena persisted, with the development of symptomatic anaemia. While colonoscopy excluded a lower gastrointestinal aetiology, CT revealed jejunitis, confirmed at capsule endoscopy. Histopathological examination of specimens obtained at single balloon enteroscopy revealed an unusual aetiology: small bowel AL-amyloidosis...
October 26, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27790444/amyloidosis-the-newer-discovered-alect2-associated-with-der7q-add-7
#19
Amrita Chakrabarti, Priyanka Samal, Joydeep Chakrabartty
Amyloidosis is characterized by pathological deposition of abnormal protein aggregates in various tissues, AL protein being the commonest. ALECT 2 is the newest protein described, having a predisposition to affect the kidneys, sometimes the liver and rarely other organs. We present a case of renal amyloid ALECT 2 due to leucocyte cell derived chemotaxin 2, a novel amyloidogenic protein. The patient presented with mild proteinuria, scattered plasma cells on bone marrow examination and altered kappa/lambda ratio with associated cytogenetic abnormality of der7q add(7)...
September 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27784371/-application-value-of-cd138-macs-fish-in-the-genetic-diagnosis-of-plasma-cell-dyscrasia
#20
Jian-Gang Mei, Han-Qing Li, Hong-Qin Cao, Jing-Jing Shao, Yong-Ping Zhai
OBJECTIVE: To investigate the cytogenetic characteristics of the various plasma cell dyscrasia using the CD138 MACS-FISH, to elucidate the application value of MACS-FISH in the genetic diagnosis of plasma cell dyscrasia, and to explore the standardization of FISH detection for plasma cell dyscrasia. METHODS: A total of 232 patients with newly diagnosed plasma cell dyscrasia were collected, including 203 cases of MM, 24 cases of AL amyloidosis and 5 cases of MGUS, whose cytogenetic abnormalities were detected by MACS-FISH, and the differences of the positive detection rates of chromosome karyotype analysis, C-FISH and MACS-FISH in MM cytogenetic abnormality were compared...
October 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
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