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Al Amyloidosis

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https://www.readbyqxmd.com/read/28808991/light-chain-al-amyloidosis-the-journey-to-diagnosis
#1
Kristen L McCausland, Michelle K White, Spencer D Guthrie, Tiffany Quock, Muriel Finkel, Isabelle Lousada, Martha S Bayliss
BACKGROUND: Light chain (AL) amyloidosis is a rare, complex disease associated with significant morbidity and mortality. Delays in diagnosis are common and may have detrimental consequences on patients' prognosis. Too little is known regarding the patient journey to diagnosis. OBJECTIVE: The objective of this study was to describe the patient-reported journey to a correct diagnosis for AL amyloidosis. METHODS: Using a mixed-methods approach, data were collected from clinician (n = 4) and patient (n = 10) interviews and a survey of community-based patients with AL amyloidosis (n = 341)...
August 14, 2017: Patient
https://www.readbyqxmd.com/read/28808514/nodular-pulmonary-amyloidosis-with-primary-pulmonary-malt-lymphoma-masquerading-as-metastatic-lung-disease
#2
Sunil Upadhaya, Mohd Baig, Basim Towfiq, Samer Al Hadidi
Nodular pulmonary amyloidosis is a very rare form of localized amyloidosis involving the lung, with very little known about its nature. It is usually associated with indolent B cell lymphoproliferative disorder and also connective tissue disorders. No definite treatment guideline exists. Many patients respond to chemotherapy with low risk of progression and a 'wait and watch' strategy is also considered a valid treatment option. In this report the authors present a case of nodular pulmonary amyloidosis with pulmonary mucosa associated lymphoid tissue (MALT) lymphoma that presented with features of metastatic malignant disease and after definitive diagnosis decided not to undergo treatment...
July 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/28807981/delineation-of-the-timing-of-second-line-therapy-post-autologous-stem-cell-transplant-in-patients-with-al-amyloidosis
#3
Yi L Hwa, Rahma Warsame, Morie A Gertz, Francis K Buadi, Martha Q Lacy, Shaji K Kumar, David Dingli, Steve R Zeldenrust, Nelson Leung, Suzanne R Hayman, Prashant Kapoor, Wilson I Gonsalves, Taxiarchis V Kourelis, Stephen Russell, Ronald S Go, Miriam A Hobbs, Amie L Fonder, S Vincent Rajkumar, Angela Dispenzieri
Among patients with immunoglobulin light chain (AL) amyloidosis, there is little consensus on when reinstitution of chemotherapy should occur. We conducted a retrospective study to evaluate the patterns of relapse or progression (R/P) and the timing of re-initiating therapy among 235 patients initially treated with ASCT at Mayo Clinic. The median time from ASCT to second-line therapy was 24.3 months. At the time of restarting therapy, median dFLC was 9.9 mg/dL (42% of diagnosis value); 32% had a dFLC< 5mg/dL; and 63% met criteria for organ R/P...
August 14, 2017: Blood
https://www.readbyqxmd.com/read/28796568/contribution-of-human-smooth-muscle-cells-to-amyloid-angiopathy-in-al-light-chain-amyloidosis
#4
Moiz Vora, Christopher G Kevil, Guillermo A Herrera
OBJECTIVE: Amyloid light-chain (AL) amyloidosis is a disease process that often compromises the peripheral vascular system and leads to systemic end-organ dysfunction. Although amyloid formation in vessel walls is a multifaceted process, the assembly of the native light chains (LCs) into amyloid fibrils is central to its pathogenesis. Recent evidence suggests that endocytosis and endolysosomal processing of immunoglobin LCs by host cells is essential to the formation of amyloid fibrils that are deposited in at least some tissues...
August 10, 2017: Ultrastructural Pathology
https://www.readbyqxmd.com/read/28790241/-amyloidoma-adjacent-to-the-thoracic-vertebral-body-developed-into-systemic-amyloid-light-chain-amyloidosis
#5
Kyoko Tanaka, Hideto Goto, Shuhei Ikeda, Nami Masumoto, Katsuya Watanabe, Motofumi Tsubakihara
Amyloidoma is very rare case of amyloidosis, and seldom develop into systemic disease. We report a case of an 82-year-old man who was referred to our hospital because of an oppressive feeling in the upper chest. Chest computed tomography showed a tumor on thoracic vertebral body. Percutaneous needle biopsy showed pathological findings of AL amyloidosis. No obvious systemic finding was confirmed, and the tumor was diagnosed as amyloidoma. After a 1-year observation, heart amyloidosis was appeared, then 6 months later he died...
August 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28758811/novel-pathologic-scoring-tools-predict-end-stage-kidney-disease-in-light-chain-al-amyloidosis
#6
Samuel Rubinstein, Robert F Cornell, Liping Du, Beatrice Concepcion, Stacey Goodman, Shelton Harrell, Sara Horst, Daniel Lenihan, David Slosky, Agnes Fogo, Anthony Langone
BACKGROUND AND OBJECTIVES: Light chain (AL) amyloidosis frequently involves the kidney, causing significant morbidity and mortality. A pathologic scoring system with prognostic utility has not been developed. We hypothesized that the extent of amyloid deposition and degree of scarring injury on kidney biopsy, could provide prognostic value, and aimed to develop pathologic scoring tools based on these features. METHODS: This is a case-control study of 39 patients treated for AL amyloidosis with biopsy-proven kidney involvement at a large academic medical center...
July 31, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28754546/comparison-of-different-stem-cell-mobilization-regimen-in-al-amyloidosis-patients
#7
Katharina Lisenko, Patrick Wuchter, Marion Hansberg, Anja Mangatter, Axel Benner, Anthony D Ho, Hartmut Goldschmidt, Ute Hegenbart, Stefan Schönland
High-dose melphalan (HDM) and autologous blood stem cell transplantation (ABSCT) is an effective treatment for transplant-eligible patients with systemic light chain (AL) amyloidosis. Whereas most centers use granulocyte colony-stimulating factor (G-CSF) alone for mobilization of peripheral blood stem cells (PBSC), the application of mobilization chemotherapy might offer specific advantages. We retrospectively analyzed 110 patients with AL amyloidosis who underwent PBSC collection. Major eligibility criteria included age <70 years and cardiac insufficiency NYHA ≤III°...
July 25, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28739313/cardiac-amyloidosis-an-update-on-pathophysiology-diagnosis-and-treatment
#8
REVIEW
Omar K Siddiqi, Frederick L Ruberg
The amyloidoses are a group of systemic diseases characterized by organ deposition of misfolded protein fragments of diverse origins. The natural history of the disease, involvement of other organs, and treatment options vary significantly based on the protein of origin. In AL amyloidosis, amyloid protein is derived from immunoglobulin light chains, and most often involves the kidneys and the heart. ATTR amyloidosis is categorized as mutant or wild-type depending on the genetic sequence of the transthyretin (TTR) protein produced by the liver...
July 13, 2017: Trends in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28737773/the-incidence-of-atrial-fibrillation-among-patients-with-al-amyloidosis-undergoing-high-dose-melphalan-and-stem-cell-transplantation-experience-at-a-single-institution
#9
M Arun, D Brauneis, G Doros, A C Shelton, J M Sloan, K Quillen, F L Ruberg, V Sanchorawala, C Varga
No abstract text is available yet for this article.
July 24, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28737209/lactate-dehydrogenase-as-a-prognostic-marker-in-al-amyloidosis-expected-or-unexpected
#10
EDITORIAL
Chor S Chim
No abstract text is available yet for this article.
July 24, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28736891/heat-induced-native-dimerization-prevents-amyloid-formation-by-variable-domain-from-immunoglobulin-light-chain-rei
#11
Marina Nawata, Hirotaka Tsutsumi, Yuta Kobayashi, Satoru Unzai, Shouhei Mine, Tsutomu Nakamura, Koichi Uegaki, Hironari Kamikubo, Mikio Kataoka, Daizo Hamada
Amyloid light chain (AL) amyloidosis is a protein-misfolding disease characterized by accumulation of immunoglobulin light chains (LCs) into amyloid fibrils. Dimerization of full length or variable domain (VL ) of LC serves to stabilize the native state and prevent the formation of amyloid fibrils. We here analyzed the thermodynamic properties of dimerization and unfolding reactions by nonamyloidogenic VL from REI LC or its monomeric Y96K mutant using sedimentation velocity and circular dichroism. The data indicate that the equilibrium shifts to native dimerization for wild type REI VL by elevating temperature due to the negative enthalpy change for dimer dissociation (-81...
July 24, 2017: FEBS Journal
https://www.readbyqxmd.com/read/28729034/prolonged-renal-survival-in-light-chain-amyloidosis-speed-and-magnitude-of-light-chain-reduction-is-the-crucial-factor
#12
Tamer Rezk, Helen J Lachmann, Marianna Fontana, Sajitha Sachchithanantham, Shameem Mahmood, Aviva Petrie, Carol J Whelan, Jennifer H Pinney, Darren Foard, Thirusha Lane, Taryn Youngstein, Ashutosh D Wechalekar, Paul Bass, Philip N Hawkins, Julian D Gillmore
Renal involvement causing progressive chronic kidney disease (CKD) is present in 70% of patients with systemic Ig light-chain (AL) amyloidosis at diagnosis. Chemotherapy that substantially suppresses free light chain production is associated with improved patient survival, but its benefit in delaying the onset of renal replacement therapy among patients who present with established advanced CKD has not been studied. To evaluate this, we studied 1000 patients enrolled in the prospective UK AL amyloidosis chemotherapy study (ALchemy)...
July 17, 2017: Kidney International
https://www.readbyqxmd.com/read/28728692/magnetic-resonance-in-transthyretin%C3%A2-cardiac-amyloidosis
#13
Ana Martinez-Naharro, Thomas A Treibel, Amna Abdel-Gadir, Heerajnarain Bulluck, Giulia Zumbo, Daniel S Knight, Tushar Kotecha, Rohin Francis, David F Hutt, Tamer Rezk, Stefania Rosmini, Candida C Quarta, Carol J Whelan, Peter Kellman, Julian D Gillmore, James C Moon, Philip N Hawkins, Marianna Fontana
BACKGROUND: Cardiac transthyretin amyloidosis (ATTR) is an increasingly recognized cause of heart failure. Cardiac magnetic resonance (CMR), with late gadolinium enhancement (LGE) and T1 mapping, is emerging as a reference standard for diagnosis and characterization of cardiac amyloidosis. OBJECTIVES: The authors used CMR with extracellular volume fraction (ECV) measurement to characterize cardiac involvement in relation to outcome in ATTR. METHODS: Subjects comprised 263 patients with cardiac ATTR corroborated by grade 2 to 3 (99m)Tc-DPD ((99m)Tc-3,3-diphosphono-1,2-propanodicarboxylic acid) cardiac uptake, 17 with suspected cardiac ATTR (grade 1 (99m)Tc-DPD), and 12 asymptomatic individuals with amyloidogenic transthyretin (TTR) mutations...
July 25, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28725274/news-in-al-amyloidosis-ash-2016-a%C3%A2-rapidly-evolving-field-of-investigation
#14
REVIEW
Hermine Agis
Amyloidosis is a rare but life-threatening protein misfolding disease. The early diagnosis and enrollment of patients into multicentre trials is of great importance, as is the need for intensive collaboration between multiple medical departments and experienced specialists. In the following review, the most interesting abstracts from the annual American Society of Hematology (ASH) meeting in 2016 are presented. The topics include the limitations of established biomarkers in risk assessment and response evaluation, the introduction of a new biomarker, the comparison of different treatment sequences and the efficacy of a multiple drug regimen in light-chain (AL) amyloidosis...
2017: Memo
https://www.readbyqxmd.com/read/28705053/cardiac-amyloidosis-pathogenesis-clinical-context-diagnosis-and-management-options
#15
Hassan Alkhawam, Darshan Patel, James Nguyen, Saumya Mariam Easaw, Mohammed Al-Sadawi, Umer Syed, Feras Zaiem, Maher Homsi, Timothy J Vittorio
Amyloidosis covers a group of disorders that can manifest in virtually any organ system in the body and is thought to be secondary to misfolding of extracellular proteins with subsequent deposition in tissues. The precursor protein that is produced in excess defines the specific amyloid type. This requires histopathological confirmation using Congo-red dye with its characteristic demonstration of green birefringence under cross-polarized light. There are three main types of amyloidosis associated with cardiac involvement: light-chain (AL), familial or senile (ATTR), and secondary (AA) amyloidosis...
August 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28701686/congestive-heart-failure-with-preserved-ejection-fraction-in-a-patient-with-light-chain-al-amyloidosis-and-multiple-myeloma
#16
Edgar Francisco Carrizales-Sepúlveda, Alejandro Ordáz-Farías, Raymundo Vera-Pineda, Mario Alberto Benavides-González, Ramiro Flores-Ramírez
BACKGROUND Amyloidosis is characterized by tissue deposition of insoluble fibrillar proteins and it affects almost every organ; there are many types and the heart can be affected in all of them. CASE REPORT Our report describes a middle-aged man who presented to the Emergency Department with congestive heart failure. Clinical, electrocardiographic, and echocardiographic findings suggested the presence of an infiltrative disease, so an abdominal fat tissue biopsy was performed. A final diagnosis of systemic amyloidosis with heart involvement was made...
July 13, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28699793/predictors-of-early-treatment-failure-following-initial-therapy-for-systemic-immunoglobulin-light-chain-amyloidosis
#17
Nidhi Tandon, Surbhi Sidana, S Vincent Rajkumar, Angela Dispenzieri, Morie A Gertz, Martha Q Lacy, Robert A Kyle, Francis K Buadi, David Dingli, Suzanne R Hayman, Amie L Fonder, Miriam A Hobbs, Wilson I Gonsalves, Prashant Kapoor, Yi Lisa Hwa, Nelson Leung, Ronald S Go, John A Lust, Stephen J Russell, Steven R Zeldenrust, Shaji K Kumar
We analysed factors predicting early treatment failure (ETF), after first-line therapy for light-chain amyloidosis (AL). AL amyloidosis patients seen at Mayo Clinic within 90 days of diagnosis, from 2006 to 2015, excluding those who died within 3 months of initial therapy, were analysed retrospectively. ETF was defined as progression requiring treatment change or death within 12 (ETF12) or 24 (ETF24) months of first-line treatment. Non-ETF included those with a follow-up of more than 12 or 24 months who had progression beyond 12 or 24 months...
July 12, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28699650/elevation-of-serum-lactate-dehydrogenase-in-al-amyloidosis-reflects-tissue-damage-and-is-an-adverse-prognostic-marker-in-patients-not-eligible-for-stem-cell-transplantation
#18
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, David Dingli, Nelson Leung, S Vincent Rajkumar, Robert A Kyle, Shaji K Kumar, Morie A Gertz
The significance of serum lactate dehydrogenase (LDH) in light chain (AL) amyloidosis has not been previously explored. We studied 1019 newly diagnosed patients and correlated the elevation of LDH above the upper limit of normal (ULN) with disease characteristics and outcome. Four hundred and nine patients had an LDH above ULN, representing 40% of the study population. Patients with an elevated LDH were older, were less likely to be male and had more extensive organ involvement compared to patients with a normal LDH...
July 12, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28698204/hematologic-relapse-in-al-amyloidosis-after-high-dose-melphalan-and-stem-cell-transplantation
#19
Sabrina Browning, Karen Quillen, J Mark Sloan, Gheorghe Doros, Shayna Sarosiek, Vaishali Sanchorawala
No abstract text is available yet for this article.
July 11, 2017: Blood
https://www.readbyqxmd.com/read/28693194/combination-of-lenalidomide-and-low-dose-dexamethasone-therapy-promotes-the-anticoagulant-activity-of-warfarin-in-patients-with-immunoglobulin-light-chain-amyloidosis
#20
Fumiaki Kitazawa, Shin-Ichi Fuchida, Fumitaka Ise, Yoko Kado, Kumi Ueda, Takatoshi Kokufu, Akira Okano, Mayumi Hatsuse, Satoshi Murakami, Yuko Nakayama, Kohji Takara, Chihiro Shimazaki
The present study aimed to evaluate the drug interactions between warfarin and combination chemotherapy with lenalidomide and low-dose dexamethasone in immunoglobulin light-chain (AL) amyloidosis patients with unstable international normalized ratios (INR). The changes to INR values over time in 3 AL amyloidosis patients treated with warfarin and a combination of lenalidomide and low-dose dexamethasone between March 2011 and February 2015 were analyzed retrospectively. The mean INR value was 1.52 prior to the combination chemotherapy, and the value increased 1...
July 2017: Oncology Letters
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