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Al Amyloidosis

Erman Öztürk, Meltem Olga Akay, Burhan Ferhanoğlu
No abstract text is available yet for this article.
March 19, 2018: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
Joshua T Byers, Xin Qing, Christopher Lo, Samuel W French, Ping Ji
Amyloidosis is a disorder characterized by the deposition of insoluble abnormal proteins in the extracellular space. It may occur as a localized lesion or as a systemic disease involving multiple organs and systems. Localized conjunctival amyloidosis is rare and is less frequently associated with systemic involvement. Although amyloidosis itself is a benign lesion involvement of multiple organs and systems is associated with poor prognosis. Diagnosis of amyloidosis is made on biopsy specimens with Congo red staining for the appearance of apple-green birefringence under polarized light microscopy...
March 15, 2018: Experimental and Molecular Pathology
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
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
Wengen Chen, Van-Khue Ton, Vasken Dilsizian
PURPOSE OF REVIEW: The two most common types of cardiac amyloidosis are caused by fibril deposits of immunoglobulin light chains (AL) and transthyretin (TTR), each with distinct prognosis and clinical management. Cardiac amyloidosis is under-recognized among heart failure patients with preserved ejection fraction (HFpEF). Bone-seeking tracers like99m Tc-PYP and99m Tc-DPD have long been used to identify cardiac amyloidosis, and more recently, to differentiate TTR from AL cardiac amyloidosis in symptomatic patients...
March 8, 2018: Current Cardiology Reports
Tulip Jhaveri, Shayna Sarosiek, Frederick L Ruberg, Omar Siddiqi, John L Berk, Vaishali Sanchorawala
No abstract text is available yet for this article.
March 8, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
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
Norman C Charles, Irina Belinsky, Habeeb Ahmad
Conjunctival amyloid is usually of the immunoglobulin light chain (AL) variety. The authors report an exceptional case of bilateral limbal amyloid deposits that were identified by mass spectrometry as keratin-related specifically regarding to basal keratinocyte keratins 5 and 14. Cytokeratin-related amyloid has been described by immunohistochemical analysis of formalin-fixed cutaneous and mucous membrane lesions but not ocular tissues. The conjunctival lesion in the right eye contained intraepithelial dyskeratotic cells that extended to the surface, causing a lacy leukoplakia on clinical examination...
March 2, 2018: Ophthalmic Plastic and Reconstructive Surgery
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
Natalia Tovar, Luis Gerardo Rodríguez-Lobato, Maria Teresa Cibeira, Laura Magnano, Ignacio Isola, Laura Rosiñol, Joan Bladé, Carlos Fernández de Larrea
OBJECTIVES: Prognosis of immunoglobulin light-chain (AL) amyloidosis depends mainly on the presence of cardiac involvement and the disease burden. A higher bone marrow plasma cell (BMPC) burden has been recognized as an adverse prognostic factor. The aim of our study was to analyze the correlation between the BMPC infiltration, clinical features and outcomes in patients with AL amyloidosis. METHODS: The clinical records of 79 patients with AL amyloidosis treated at a single institution...
February 26, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
M Pick, V Vainstein, N Goldschmidt, D Lavie, D Libster, A Gural, S Grisariu, B Avni, D Ben Yehuda, M E Gatt
OBJECTIVE: Daratumumab is a promising new anti-myeloma agent. We report a single center "real world" series of multiple myeloma (MM) and amyloidosis (AL) patients treated with daratumumab. METHODS: Forty-one patients were included: 7 second line MM, 30 heavily pretreated (median number of therapies of 5) advanced MM, and 4 with AL. RESULTS: Second line patients and advanced AL showed high rate of durable overall responses. However, advanced MM patients had a dismal prognosis with an ORR of 36%, and a short median progression free and overall survival of 2...
February 17, 2018: European Journal of Haematology
L Li, X J Duan, Y Sun, Y Lu, H Y Xu, Q Z Wang, H Y Wang
Objective: To evaluate the sensitivity and specificity of immunohistochemistry (IHC) in the classification of cardiac amyloidosis on endomyocardial biopsy (EMB) and heart allograft. Methods: Twenty cardiac tissues from 19 patients at Fuwai Hospital from January, 1990 to April, 2017 with histopathologic features of amyloidosis and Congo red staining positivity were included. IHC was performed with monoclonal antibodies against AA amyloid and polyclonal antibodies against transthyretin (ATTR), λ-light chain (AL-λ), κ-light chain (AL-κ), ApoAⅠ, ApoAⅡ, ApoA Ⅳ and β(2)-microglobin...
February 8, 2018: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
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
Francesco Cappelli, Chiara Gallini, Egidio Natalino Costanzo, Francesca Tutino, Alfonso Ciaccio, Luca Vaggelli, Simone Bartolini, Sofia Morini, Raffaele Martone, Paola Angelotti, Sabrina Frusconi, Carlo Di Mario, Federico Perfetto
BACKGROUND: Full body scintigraphy using bone tracers plays an important role in defining the type of amyloidosis and in diagnosing the heart involvement (cardiac amyloidosis, CA). No study has been conducted to explore lung retention (LR) in CA and its correlation to heart retention (HR).We evaluated LR in patients undergoing 99mTc-HMDP scintigraphy during evaluation for suspected CA. METHODS AND RESULTS: We enrolled 93 suspected CA patients. Patients underwent a complete diagnostic work up...
March 1, 2018: International Journal of Cardiology
Anum Bilal, Paul Der Mesropian, Franklin Lam, Gulvahid Shaikh
Amyloidosis is a systemic illness characterized by the extracellular deposition of abnormal proteins in body tissues and organs. In addition to renal involvement, amyloidosis can also present with a variety of skin manifestations, though rarely with alopecia. Sixteen cases of alopecia secondary to systemic amyloidosis are reported. There is one reported case that presented with alopecia universalis. We report a case of a 68-year-old woman presenting with alopecia universalis, rapid decline in kidney function, and nephrotic syndrome who was found to have multiple myeloma-associated AL amyloidosis (immunoglobulin light chain)...
January 2018: Journal of Investigative Medicine High Impact Case Reports
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
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)
Efstathios Kastritis, Ioannis Papassotiriou, Giampaolo Merlini, Paolo Milani, Evangelos Terpos, Marco Basset, Athanasios Akalestos, Francesca Russo, Erasmia Psimenou, Filia Apostolakou, Maria Roussou, Maria Gavriatopoulou, Evangelos Eleutherakis Papaiakovou, Despina Fotiou, Dimitrios C Ziogas, Elektra Papadopoulou, Constantinos Pamboucas, Meletios A Dimopoulos, Giovanni Palladini
Growth differentiation factor-15 (GDF-15) improves prognostication in patients with cardiovascular disorders in addition to conventional cardiac markers (NT-proBNP, troponins) and has shown prognostic value in patients with renal diseases. In patients with AL amyloidosis cardiac involvement is the major determinant of prognosis and cardiac markers define prognosis, while biomarkers of renal involvement stratify renal risk. We explored the prognostic importance of serum levels of GDF-15 in patients with AL amyloidosis, in two independent cohorts...
January 31, 2018: Blood
Michiko Aoki, Dedong Kang, Akira Katayama, Naomi Kuwahara, Shinya Nagasaka, Yoko Endo, Mika Terasaki, Shinobu Kunugi, Yasuhiro Terasaki, Akira Shimizu
BACKGROUND: Liquid chromatography-tandem mass spectrometry (LC-MS/MS) has recently been utilized to accurately detect the amyloid proteins of renal amyloidosis. The present study investigated the optimal procedures for analyzing samples by LCMS/MS, and the advantage of using this technique to diagnosis renal amyloidosis. METHODS: To detect amyloid proteins, laser microdissected glomeruli from AL (n = 13) or AA (n = 10) renal amyloidosis patients were digested and analyzed by LCMS/MS...
January 25, 2018: Clinical and Experimental Nephrology
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
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