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Al Amyloidosis

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https://www.readbyqxmd.com/read/29321425/elevation-of-plasmin-%C3%AE-2-plasmin-inhibitor-complexes-in-patients-with-al-amyloidosis
#1
Kanji Miyazaki, Kenshi Suzuki
No abstract text is available yet for this article.
January 11, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29319035/a-rare-case-of-bronchial-elastosis-an-unusual-presentation-of-an-unexpandable-lung
#2
Raghav Gupta, Hassan Patail, Mohammad R Al-Ajam
Endobronchial obstruction is a known cause of an unexpandable lung. Endobronchial lesions are usually malignant, however benign cause like hemartoma, lipoma, amyloidosis and neuroendocrine tumors are known. We, hereby present the rare cause of bronchial elastosis presenting as right lower lobe lung collapse.
January 2018: Lung India: Official Organ of Indian Chest Society
https://www.readbyqxmd.com/read/29303358/treatment-patterns-and-health-care-resource-utilization-among-patients-with-relapsed-refractory-systemic-light-chain-amyloidosis
#3
Parameswaran Hari, Huamao Mark Lin, Carl V Asche, Jinma Ren, Candice Yong, Katarina Luptakova, Douglas V Faller, Vaishali Sanchorawala
BACKGROUND: Treatment for patients with systemic light chain (AL) amyloidosis remains challenging. Our study aims to describe treatment patterns for both newly diagnosed and relapsed/refractory AL (RRAL) amyloidosis, and to assess clinical outcomes, healthcare costs, and resource utilization during the first year following a diagnosis of RRAL amyloidsis. METHODS: This was a retrospective observational study of adult patients with AL amyloidosis using the US Optum administrative claims data during 1/1/2008 to 6/30/2015...
January 5, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29303069/unfolding-cardiac-amyloidosis-from-pathophysiology-to-cure
#4
Klemens Ablasser, Nicolas Verheyen, Theresa Glantschnig, Giulio Agnetti, Peter P Rainer
Deposition of amyloidogenic proteins leading to the formation of amyloid fibrils in the myocardium cause cardiac amyloidosis. Although any form of systemic amyloidosis can affect the heart, light-chain (AL) or transthyretin amyloidosis (ATTR) account for the majority of diagnosed cardiac amyloid deposition. The extent of cardiac disease independently predicts mortality. The reversal or arrest of adverse cardiac remodeling is the target of current therapies, as cardiac-related mortality worsens prognosis in patients where the underlying systemic amyloidosis was successfully treated...
January 4, 2018: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/29298704/the-prognostic-value-of-t1-mapping-and-late-gadolinium-enhancement-cardiovascular-magnetic-resonance-imaging-in-patients-with-light-chain-amyloidosis
#5
Lu Lin, Xiao Li, Jun Feng, Kai-Ni Shen, Zhuang Tian, Jian Sun, Yue-Ying Mao, Jian Cao, Zheng-Yu Jin, Jian Li, Joseph B Selvanayagam, Yi-Ning Wang
BACKGROUND: Cardiac impairment is associated with high morbidity and mortality in immunoglobulin light chain (AL) type amyloidosis, for which early identification and risk stratification is vital. For myocardial tissue characterization, late gadolinium enhancement (LGE) is a classic and most commonly performed cardiovascular magnetic resonance (CMR) parameter. T1 mapping with native T1 and extracellular volume (ECV) are recently developed quantitative parameters. We aimed to investigate the prognostic value of native T1, ECV and LGE in patients with AL amyloidosis...
January 3, 2018: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/29282382/massive-upper-and-lower-gi-bleed-from-simultaneous-primary-al-amyloidosis-of-the-stomach-and-transverse-colon-in-a-patient-with-multiple-myeloma
#6
Zubair Khan, Umar Darr, Anas Renno, Abhinav Tiwari, Aijaz Sofi, Ali Nawras
Symptomatic primary (amyloid light-chain or AL) amyloidosis of the gastrointestinal (GI) tract is very rare. Most of the patients with symptomatic involvement of the GI tract present with altered motility, malabsorption, or bleeding. We report a case of gastric and colonic amyloidosis on anticoagulation presenting with massive upper and lower GI bleeding. A 67-year-old lady known to have multiple myeloma and AL amyloidosis on rivaroxaban presented with massive upper GI bleeding. Esophagogastroduodenoscopy showed a mass lesion (3 × 7 cm) located along the greater curvature in the body/antrum with active bleeding...
September 2017: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/29260587/right-ventricular-longitudinal-strain-a-tool-for-diagnosis-and-prognosis-in-light-chain-amyloidosis
#7
Charles Uzan, Olivier Lairez, Pascale Raud-Raynier, Rodrigue Garcia, Bruno Degand, Luc P Christiaens, Michaela B Rehman
OBJECTIVES: Light-chain (AL) amyloidosis can lead to an infiltrative cardiomyopathy with increased wall thickness (IWT) of very poor prognosis. Our primary aim was to analyse the right ventricle (RV) in patients with IWT to discriminate AL amyloidosis from IWT due to hypertrophic cardiomyopathy (HCM) or to arterial hypertension (HTN). Our secondary aim was to assess if RV dysfunction predicts overall mortality in cardiac AL amyloidosis. METHODS: We retrospectively and consecutively compared clinical, biological and echocardiographic data of 315 patients with IWT: 105 biopsy-proven AL amyloidosis patients, 105 patients with HCM and 105 patients with HTN...
December 20, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29249671/gastric-submucosal-haematoma-caused-by-an-amyloidoma-in-the-setting-of-multiple-myeloma
#8
Stella C Pak, John-Phillip T Markovic
This is a case of a 67-year-old woman with a history of multiple myeloma with amyloidosis who presented with massive hematemesis. Emergent upper endoscopy revealed a mass on the greater curvature of the stomach, which measured approximately 3 cm in width and 7 cm in length. The patient underwent a wedge resection of the gastric mass without complication. Microscopic examination of the gastric mass revealed amorphous deposits that were congophilic in nature and stained positive with Alcian blue. These findings are consistent with amyloidosis...
December 14, 2017: Arab Journal of Gastroenterology: the Official Publication of the Pan-Arab Association of Gastroenterology
https://www.readbyqxmd.com/read/29241368/gastrointestinal-perforation-in-light-chain-amyloidosis-in-the-era-of-novel-agent-therapy-a-case-series-and-review-of-the-literature
#9
Adir Shaulov, Irit Avivi, Yael Cohen, Adrian Duek, Merav Leiba, Moshe E Gatt
Gastrointestinal (GI) perforation is remarkably rare in patients with light chain (AL) amyloidosis and has not yet been reported in patients with AL amyloidosis treated with novel agents. Only 24 cases of GI perforation have previously been reported in the setting of AL amyloidosis of which 15 had available information in English. All 15 did not receive novel agent therapy and six died early after experiencing GI perforation. This study reports the characteristics and outcome of AL patients that developed GI perforation in the era of novel agent treatment...
December 14, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29233648/left-atrial-dysfunction-in-light-chain-cardiac-amyloidosis-and-hypertrophic-cardiomyopathy-a-comparative-three-dimensional-speckle-tracking-echocardiographic-analysis-from-the-magyar-path-study
#10
Dóra Földeák, Árpád Kormányos, Péter Domsik, Anita Kalapos, Györgyike Á Piros, Nóra Ambrus, Zénó Ajtay, Róbert Sepp, Zita Borbényi, Tamás Forster, Attila Nemes
INTRODUCTION: While cardiac amyloidosis (CA) is a rare systemic disease characterized by extracellular deposition of protein-derived fibrils, hypertrophic cardiomyopathy (HCM) is histopathologically characterized by myocyte hypertrophy and disarray, interstitial fibrosis, and small intramural coronary arteriole dysplasia. The aim of the present study was to compare left atrial (LA) volumetric and functional characteristics between light-chain (AL) CA and HCM by three-dimensional (3D) speckle-tracking echocardiography (STE)...
December 9, 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/29226427/case-report-treatment-of-light-chain-amyloidosis-with-daratumumab-monotherapy-in-two-patients
#11
Charlotte Gran, Gösta Gahrton, Evren Alici, Hareth Nahi
Immunoglobulin light-chain amyloidosis (AL) affects multiple organs, most prominently the kidney and the heart. Renal and cardiac impairment are both associated with poor prognosis.(1) Typical treatment regimens for AL include proteasome inhibitors, alkylating agents, and steroids as well as autologous stem cell transplantation (ASCT) for younger, fit patients. Complete response after treatment is associated with a better outcome and can be measured by free light chain (FLC) reduction.(2, 3) Monoclonal antibodies such as daratumumab (Dara, human IgG1 anti-CD38) have shown promising efficacy for the treatment of relapsed and refractory multiple myeloma...
December 11, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/29224328/-clinical-characteristics-and-prognostic-analysis-of-monoclonal-igm-related-al-amyloidosis
#12
Y Y Mao, J Feng, Q Meng, K N Shen, X X Cao, D B Zhou, J Li
No abstract text is available yet for this article.
November 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29223373/adverse-prognostic-factors-for-morbidity-and-mortality-during-peripheral-blood-stem-cell-mobilization-in-patients-with-light-chain-amyloidosis
#13
Jason C Yeh, Brandon R Shank, Denái R Milton, Muzaffar H Qazilbash
Patients with immunoglobulin light chain (AL) amyloidosis undergoing peripheral blood hematopoietic stem cell (PBSC) mobilization for autologous hematopoietic stem cell transplantation (auto-HCT) can experience significant morbidity and mortality. The purpose of this study was to describe the adverse events and identify prognostic factors associated with the development of morbidity and mortality in patients with AL amyloidosis who had begun PBSC mobilization for auto-HCT. A retrospective study was performed in 101 consecutive patients with AL amyloidosis who underwent PBSC mobilization for auto-HCT between January 2006 and December 2013...
December 6, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29222812/spheroid-type-of-al-amyloid-deposition-associated-with-colonic-adenocarcinoma-a-case-report-with-literature-review
#14
Moon Joo Kim, Zulfia McCroskey, Yingchao Piao, Anna Belcheva, Luan Truong, Paul J Kurtin, Jae Y Ro
We report a colonic adenocarcinoma associated with diffuse submucosal deposition of a peculiar spheroid-type amyloid identified in the colon, terminal ileum, and appendix. A 65-year-old woman with past medical histories of hypertension, and chronic obstructive pulmonary disease, presented to the emergency room with cramping abdominal pain and nausea. A computed tomography (CT) scan of abdomen showed right colonic volvulus. Emergency right hemicolectomy was performed. The specimen showed colonic adenocarcinoma with focal submucosal invasion (pT1) arising from a villotubular adenoma...
December 8, 2017: Pathology International
https://www.readbyqxmd.com/read/29222231/al-amyloidosis-from-molecular-mechanisms-to-targeted-therapies
#15
REVIEW
Giampaolo Merlini
Systemic amyloidosis is caused by misfolding and extracellular deposition of circulating proteins as amyloid fibrils, resulting in the dysfunction of vital organs. The most common systemic amyloidosis, light-chain (AL) amyloidosis, is caused by misfolded light chains produced by a small, dangerous B-cell clone. The process of amyloid formation, organ targeting, and damage is multifaceted and, after disease initiation, the complexity of the downstream pathogenic cascade increases, rendering its control a challenge...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29219632/budd-chiari-syndrome-associated-with-al-amyloidosis-a-coagulation-paradox
#16
Guilherme Grossi Lopes Cançado, Luciana Costa Faria, Fernanda Maria Farage Osório, Paula Vieira Teixeira Vidigal, Cláudia Alves Couto, Teresa Cristina de Abreu Ferrari
No abstract text is available yet for this article.
December 8, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29217108/improved-outcomes-after-heart-transplantation-for-cardiac-amyloidosis-in-the-modern-era
#17
Arnt V Kristen, Michael M Kreusser, Patrick Blum, Stefan O Schönland, Lutz Frankenstein, Andreas O Dösch, Benjamin Knop, Matthias Helmschrott, Bastian Schmack, Arjang Ruhparwar, Ute Hegenbart, Hugo A Katus, Philip W J Raake
BACKGROUND: Cardiac amyloidosis, caused most commonly by deposition of light chain (AL) or transthyretin (ATTR) type fibrils, has an extremely poor prognosis. In this retrospective single-center study, we evaluated temporal trends in survival after heart transplantation for cardiac amyloidosis. METHODS: We analyzed 48 patients with cardiac amyloidosis (AL, n = 32; familial ATTR, n = 16) who underwent heart transplantation from May 2002 to March 2017. Patients were analysed in 2 periods, Era 1 (2002- 2007) and Era 2 (2008- 2017), separated by altered patient selection in both, AL and ATTR amyloidosis, and changed chemotherapy regimens for AL amyloidosis...
November 15, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29212969/-analysis-of-long-term-survivors-with-cardiac-al-amyloidosis
#18
Kumiko Kagawa, Yusaku Maeda, Masahiro Oura, Kimiko Sogabe, Hikaru Fujino, Mamiko Takahashi, Tomoko Maruhashi, Masami Iwasa, Kengo Udaka, Takeshi Harada, Takayuki Ise, Shiro Fujii, Shingen Nakamura, Hirokazu Miki, Shusuke Yagi, Kyoko Takeuchi, Shuji Ozaki, Masahiro Abe
Cardiac AL amyloidosis (CA) is generally known as a severe disease with very poor prognosis. Here we retrospectively examined seven patients with CA in our cohort who achieved long-term survival. All six patients who underwent high-dose melphalan and autologous stem cell transplantation (ASCT) survived for >3 years, whereas four patients survived for >5 years. Patients who underwent ASCT had prompt hematological responses, and five patients showed organ responses. ASCT helps to achieve a quick and deep hematological response required for long-term survival in patients with CA...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29212103/recurrent-diffuse-gastric-bleeding-as-a-leading-symptom-of%C3%A2-gastrointestinal-al-amyloidosis
#19
Caspar Franck, Marino Venerito, Jochen Weigt, Albert Roessner, Peter Malfertheiner
Amyloidosis is a rare disease (incidence about 0.8/100 000) characterized by extracellular tissue deposition of fibrils composed of low molecular weight subunits of a variety of serum proteins. Clinical manifestations are largely determined by the type of precursor protein, the tissue distribution and the amount of amyloid deposition. Gastrointestinal (GI) manifestations of amyloidosis are even more uncommon (3 % of all amyloidosis patients). Symptoms of GI amyloidosis are nonspecific, heterogeneous, and include weight loss, GI bleeding, heartburn, early satiety, diarrhea and abdominal pain...
December 2017: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/29200282/incomplete-refolding-of-antibody-light-chains-to-non-native-protease-sensitive-conformations-leads-to-aggregation-a-mechanism-of-amyloidogenesis-in-patients
#20
Gareth J Morgan, Grace A Usher, Jeffery W Kelly
Genetic, biochemical, and pharmacologic evidence supports the hypothesis that conformationally altered or misfolded protein states enable aggregation and cytotoxicity in the systemic amyloid diseases. Reversible structural fluctuations of natively folded proteins are involved in the aggregation of many degenerative disease associated proteins. Herein, we use antibody light chains (LCs) that form amyloid fibrils in AL amyloidosis to consider an alternative hypothesis of amyloidogenesis: that transient unfolding and incomplete extracellular refolding of secreted proteins can lead to metastable, alternatively folded states that are more susceptible to aggregation or to endoproteolysis that can release aggregation-prone fragments...
December 19, 2017: Biochemistry
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