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https://www.readbyqxmd.com/read/28632471/lenalidomide-as-a-treatment-for-relapsed-al-amyloidosis-in-an-hiv-positive-patient
#1
Johanna Denman, Kaveh Manavi, Mark Cook
We present a case of an HIV-positive man with systemic immunoglobulin light chain (AL) amyloid with cardiac involvement. At relapse, he was treated with lenalidomide and dexamethasone having previously developed autonomic neuropathy with bortezomib-based chemotherapy. The patient achieved a serological complete response with symptomatic improvement. After 11 cycles, lenalidomide was discontinued due to extensive ischaemia of the gastrointestinal tract. The patient remains symptomatically stable with normal levels of serum-free light chains 11 months after the treatment was discontinued...
January 1, 2017: International Journal of STD & AIDS
https://www.readbyqxmd.com/read/28632419/in-vitro-co-expression-of-human-amyloidogenic-immunoglobulin-light-and-heavy-chain-proteins-a-relevant-cell-based-model-of-al-amyloidosis
#2
Elena S Klimtchuk, Tatiana B Prokaeva, Brian H Spencer, Olga Gursky, Lawreen H Connors
Immunoglobulin (Ig) light chain (LC) amyloidosis (AL) is characterized by the overproduction and tissue deposition of monoclonal LC in various organs and tissues. The plasma circulating monoclonal LC is believed to be the precursor of the deposited protein and in vitro studies aimed at understanding AL pathobiology have mainly focused on LC and its variable domain. While 33% of patients have free circulating monoclonal LC, ∼40% feature LC complexed to heavy chain (HC) forming a monoclonal intact Ig; the significance of free vs...
June 20, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28617941/association-of-igg4-and-free-light-chain-with-idiopathic-pleural-effusion
#3
Yoriyuki Murata, Keisuke Aoe, Yuka Mimura-Kimura, Tomoyuki Murakami, Keiji Oishi, Tsuneo Matsumoto, Hiroshi Ueoka, Kazuto Matsunaga, Masafumi Yano, Yusuke Mimura
The cause of pleural effusion remains uncertain in approximately 15% of patients despite exhaustive evaluation. As recently described IgG4-related disease is a fibroinflammatory disorder that can affect various organs including the lungs, we investigate whether idiopathic pleural effusion includes IgG4-associated etiology. Between 2000 and 2012, we collected 830 pleural fluid samples and reviewed 35 patients with pleural effusions undiagnosed after pleural biopsy at Yamaguchi-Ube Medical Center. Importantly, IgG4 immunostaining revealed infiltration of IgG4-positive plasma cells in the pleura of 12 patients (34%, IgG4+ group)...
June 15, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28617494/spectral-and-photochemical-properties-of-hybrid-organic-inorganic-nanosystems-based-on-cds-quantum-dots-and-merocyanine-ligands
#4
Oleg V Chashchikhin, Mikhail F Budyka
The spectral and photochemical properties of hybrid organic-inorganic nanosystems (HNSs) were investigated. HNS consisted of CdS quantum dots (QDs) functionalized with ligands containing the isothiouronium anchor group linked by a polymethylene chain with photochromic merocyanine (MC). The HNS synthesis was carried out via a microwave-assisted one-pot technique. Energy transfer from the QDs to MC in the HNS was observed and resulted in QD fluorescence quenching and MC sensitization. Compared to the free MC, trans-cis photoisomerization of MC in the HNS was suppressed and its photodestruction was accelerated...
June 15, 2017: Photochemical & Photobiological Sciences
https://www.readbyqxmd.com/read/28617363/-diagnostic-value-of-immunoglobulin-free-light-chains-at-the-debut-of-multiple-sclerosis
#5
V D Nazarov, G S Makshakov, A V Mazing, E A Surkova, V S Krasnov, M V Shumilina, N A Totolyan, E P Evdoshenko, S V Lapin, V L Emanuel, A A Skoromets
AIM: To evaluate the diagnostic value of determination of free immunoglobulin light chains (IgG) in the debut of multiple sclerosis (MS). MATERIAL AND METHODS: Data from 226 patients, including 111 patients with clinically isolated syndrome with conversion to multiple sclerosis within the first 2 years of the disease (group 1), 49 patients with clinically isolated syndrome who did not develop multiple sclerosis within the first 2 years of the disease (group 2), 20 patients with other inflammatory diseases of the central nervous system (group 3) were analyzed...
2017: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://www.readbyqxmd.com/read/28606401/monoclonal-gammopathy-with-both-nemaline-myopathy-and-amyloid-myopathy
#6
Min Wang, Lin Lei, Hai Chen, Li Di, Mi Pang, Yan Lu, Lu Lu, Xin-Ming Shen, Yuwei Da
Monoclonal gammopathies due to plasma cell dyscrasias can induce diverse rare neuromuscular disorders. Deposition of monoclonal antibody light chains in skeletal muscle causes amyloid myopathy. Monoclonal gammopathy is occasionally associated with sporadic late-onset nemaline myopathy. Here we report a monoclonal gammopathy patient with both sporadic late-onset nemaline myopathy and amyloid myopathy. The diagnoses were based on immunofixation electrophoresis of urine, and serum for free light chain assay, Congo red staining and Thioflavin S staining of muscle biopsies, as well as immunohistochemical staining and electron-microscopic observation...
May 11, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28605324/sulfation-of-the-extracellular-polysaccharide-produced-by-the-king-oyster-culinary-medicinal-mushroom-pleurotus-eryngii-agaricomycetes-and-its-antioxidant-properties-in-vitro
#7
Xuewei Jia, Chong Wang, Yanhong Bai, Jinwei Yu, Chunping Xu
Extracellular polysaccharide from Pleurotus eryngii was sulfated; the optimum reaction conditions used pyridine as the reaction solvent, a temperature of 80°C, and a reaction time of 90 minutes. The structure and antioxidant activity of the sulfated and original polysaccharides (designated as SF-1 and Fr-1, respectively) were investigated. The main functional groups of polysaccharides were determined by Fourier transform infrared, and the chain conformation was determined by size-exclusion chromatography with laser light scattering and viscometry...
2017: International Journal of Medicinal Mushrooms
https://www.readbyqxmd.com/read/28601282/kflc-index-utility-in-multiple-sclerosis-diagnosis-further-confirmation
#8
M Pieri, M Storto, S Pignalosa, R Zenobi, F Buttari, S Bernardini, D Centonze, M Dessi
The Multiple Sclerosis (MS) diagnosis is based on dissemination of focal lesions in time and space. The free light chains (FLCs) determination might be a sensitive alternative to oligoclonal bands assay. The study aim was to redefine sensitivity, specificity of the kFLC Index cut-off. We analyzed serum and cerebrospinal fluid of 176 patients, with different neurological disorders. We obtained a cut off of 12,3 for kFLC Index with a sensitivity and specificity of 93% and 100% respectively. Our data confirm that the kFLC Index is a valid tool in the diagnosis of MS...
August 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28596293/the-uncoupling-of-autophagy-and-zinc-homeostasis-in-airway-epithelial-cells-as-a-fundamental-contributor-to-copd
#9
Eugene Roscioli, Hai Bac Tran, Hubertus Jersmann, Phan Tien Nguyen, Emily Hopkins, Susan Elizabeth Lester, Nigel Farrow, Peter D Zalewski, Paul N Reynolds, Sandra Hodge
The proper regulation of Zinc (Zn) trafficking proteins and the cellular distribution of Zn is critical for the maintenance of autophagic processes. However, there have been no studies which have examined Zn dyshomeostasis and the disease-related modulation of autophagy observed in the airways afflicted with COPD. We hypothesized that dysregulated autophagy in airway epithelial cells (AEC) is related to Zn dysregulation in cigarette smoke (CS)-induced COPD. We applied a human ex vivo air-liquid interface model, a murine model of smoke-exposure, and human lung tissues, and investigated Zn, ZIP1 and ZIP2 Zn-influx proteins, autophagy (Microtubule-associated 1A/1B-light chain-3 (LC3), Beclin-1), autophagic flux (Sequestosome), apoptosis (Bcl2; X-Linked Inhibitor of Apoptosis (XIAP), Poly (ADP)-ribose Polymerase (PARP)), and inflammation (TSLP, RANTES, and IL-1β)...
June 8, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28575180/performance-evaluation-of-serum-free-light-chain-analysis-nephelometry-vs-turbidimetry-monoclonal-vs-polyclonal-reagents
#10
Anne-Sophie Messiaen, Maxime M W De Sloovere, Paul-Emile Claus, Martine Vercammen, Lieve Van Hoovels, Olivier Heylen, Johan Debrabandere, Hilde Vanpoucke, Dieter De Smet
Objectives: Free light chain (FLC) measurement gained a lot of interest for diagnostic workup of monoclonal gammopathy. Methods: We evaluated the performance of turbidimetric polyclonal Freelite (The Binding Site, Birmingham, UK) assays on Cobas 6000 (Roche Diagnostics, Rotkreuz, Switzerland) and nephelometric monoclonal N Latex (Siemens Healthcare Diagnostics, Marburg, Germany) assays on BN ProSpec (Dade Behring, Deerfield, IL) vs established nephelometric Freelite assays on BN ProSpec...
June 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28574076/bushy-sphere-dendrites-with-husk-shaped-branches-axially-spreading-out-from-the-core-for-photo-catalytic-oxidation-remediation-of-toxins
#11
Mohamed A Shenashen, Satoshi Kawada, Mahmoud M Selim, Wafaa M Morsy, Hitoshi Yamaguchi, Abdulaziz A Alhamid, Naoki Ohashi, Izumi Ichinose, Sherif A El-Safty
This work describes densely interlinked bushy "tree-like chains" characterized by neatly branched sphere dendrites (bushy sphere dendrites, BSD) with long fan-like, husk-shaped branching paths that extend longitudinally from the core axis of the {110}-exposed plane. We confirmed that the hierarchical dendrite surfaces created bowls of swirled caves along the tree-tube in the mat-like branches. These surfaces had high-index catalytic site facets associated with the formation of ridges/defects on the dominant {110}-top-cover surface...
June 2, 2017: Nanoscale
https://www.readbyqxmd.com/read/28573706/diagnosis-and-monitoring-for-light-chain-only-and-oligosecretory-myeloma-using-serum-free-light-chain-tests
#12
Jennifer L J Heaney, John P Campbell, Anne E Griffin, Jane Birtwistle, Meena Shemar, J Anthony Child, Walter M Gregory, David A Cairns, Gareth Morgan, Graham Jackson, Mark T Drayson
This study aims to guide the integration of serum free light chain (sFLC) tests into clinical practice, including a new rapid test (Seralite(®) ). Blood and urine analysis from 5573 newly diagnosed myeloma patients identified 576 light chain only (LCO) and 60 non-secretory (NS) cases. Serum was tested by Freelite(®) and Seralite(®) at diagnosis, maximum response and relapse. 20% of LCO patients had urine FLC levels below that recommended for measuring response but >97% of these had adequate sFLC levels (oligosecretory)...
June 1, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28571339/unexpected-impact-of-irreversible-adsorption-on-thermal-expansion-adsorbed-layers-are-not-that-dead
#13
Marie-Luise Braatz, Leslie Infantas Meléndez, Michele Sferrazza, Simone Napolitano
We investigated the impact of irreversible adsorption on the mechanisms of thermal expansion of 1D confined polymer layers. For spincoated films (polystyrene on aluminum) of constant thickness, the thermal expansion coefficient of the melt drops upon annealing following the kinetics of irreversible adsorption of the chains onto the supporting substrate, while the thermal expansion of the glass is annealing invariant. These perturbations are explained in terms of the reduction in free volume content, upon immobilization of monomers onto the substrate...
May 28, 2017: Journal of Chemical Physics
https://www.readbyqxmd.com/read/28561668/established-and-novel-prognostic-biomarkers-in-multiple-myeloma
#14
Mark Bustoros, Tarek H Mouhieddine, Alexandre Detappe, Irene M Ghobrial
Multiple myeloma (MM) is an incurable plasma cell malignancy characterized by notable interpatient heterogeneity. There have been important advances in therapy and overall survival, but some patients with high-risk features still have poor survival rates. Therefore, accurate identification of this subset of patients has been integral to improvement of patient outcome. During the last few years, cytogenetics, gene expression profiling, MRI and PET/CT, as well as serum free light chain assays have been used as accurate biomarkers to better characterize the diverse course and outcome of the disease...
2017: American Society of Clinical Oncology Educational Book
https://www.readbyqxmd.com/read/28556363/photocontrolled-living-polymerization-systems-with-reversible-deactivations-through-electron-and-energy-transfer
#15
REVIEW
Sivaprakash Shanmugam, Jiangtao Xu, Cyrille Boyer
Recently, visible-light-regulated polymerization has been gaining popularity, as it opens a range of new opportunities for the synthesis of functional polymers and materials. Here, the most recent developments in this field are summarized, which is the use of photocatalysts and catalyst-free approaches to mediate polymerization upon photoexcitation. These catalysts can transfer an electron or energy to activate an initiator. The recent achievements in light-regulated atom-transfer radical polymerization, reversible addition-fragmentation chain-transfer polymerization, ring-opening metathesis polymerization, cobalt-mediated radical polymerization, iodine-mediated radical polymerization, and living cationic polymerization are reviewed...
May 26, 2017: Macromolecular Rapid Communications
https://www.readbyqxmd.com/read/28550043/al-amyloidosis-patients-with-low-amyloidogenic-free-light-chain-levels-at-first-diagnosis-have-an-excellent-prognosis
#16
Tobias Dittrich, Tilmann Bochtler, Christoph Kimmich, Natalia Becker, Anna Jauch, Hartmut Goldschmidt, Anthony D Ho, Ute Hegenbart, Stefan O Schönland
The difference between involved minus uninvolved serum free light chains (dFLC) has been established as invaluable hematologic parameter in systemic light chain amyloidosis (AL). However, patients with initial dFLC below 50mg/L are currently deemed not evaluable for response to therapy. Therefore, we aimed to characterize this subgroup and to define novel hematologic response parameters. We retrospectively analyzed 783 AL patients newly diagnosed at our center between 2002 and 2016. Patients with dFLC<50 showed a smaller bone marrow plasmacytosis (7% vs...
May 26, 2017: Blood
https://www.readbyqxmd.com/read/28550039/a-phase-1-2-study-of-the-oral-proteasome-inhibitor-ixazomib-in-relapsed-or-refractory-light-chain-al-amyloidosis
#17
Vaishali Sanchorawala, Giovanni Palladini, Vishal Kukreti, Jeffrey A Zonder, Adam D Cohen, David C Seldin, Angela Dispenzieri, Arnaud Jaccard, Stefan O Schönland, Deborah Berg, Huyuan Yang, Neeraj Gupta, Ai-Min Hui, Raymond L Comenzo, Giampaolo Merlini
This phase 1/2 study assessed the safety, tolerability, and preliminary efficacy of the oral proteasome inhibitor (PI) ixazomib in patients with relapsed/refractory AL amyloidosis (RRAL). Ixazomib was administered to adult patients with RRAL after ≥1 prior line of therapy (including bortezomib) on days 1, 8, and 15 of 28-day cycles, for up to 12 cycles. Patients with less than partial response after 3 cycles received oral dexamethasone (40 mg, days 1-4) from cycle 4. A 3+3 dose-escalation phase was followed by 2 expansion cohorts (PI-naïve and PI-exposed patients) at the maximum tolerated dose (MTD)...
May 26, 2017: Blood
https://www.readbyqxmd.com/read/28546143/patients-with-light-chain-al-amyloidosis-and-low-free-light-chain-burden-have-distinct-clinical-features-and-outcome
#18
Paolo Milani, Marco Basset, Francesca Russo, Andrea Foli, Giampaolo Merlini, Giovanni Palladini
The validated criteria of hematologic response in AL amyloidosis are based on the measurement of circulating free light chains (FLC). Patients with a difference between involved and uninvolved FLC (dFLC) <50 mg/L cannot be assessed for response and are excluded from clinical trials. We compared the clinical characteristics and outcome of 203 newly diagnosed patients with dFLC<50 mg/L (low-dFLC) with 866 patients with measurable dFLC (high-dFLC), evaluated between 2004 and 2015. Heart involvement was significantly less common and less advanced in the low-dFLC group (43% vs...
May 25, 2017: Blood
https://www.readbyqxmd.com/read/28546074/outcomes-from-autologous-hematopoietic-cell-transplantation-versus-chemotherapy-alone-for-the-management-of-light-chain-al-amyloidosis
#19
Oluchi Oke, Tarsheen Sethi, Stacey Goodman, Sharon Phillips, Ilka Decker, Samuel Rubinstein, Beatrice Concepcion, Sarah Horst, Madan Jagasia, Adetola Kassim, Shelton L Harrell, Anthony Langone, Daniel Lenihan, Kyle T Rawling, David Slosky, R Frank Cornell
Light chain amyloidosis (AL) results in tissue deposition of misfolded proteins causing organ dysfunction. In an era of modern therapies such as bortezomib, reassessment of the benefit of autologous hematopoietic cell transplantation (AHCT) should be considered. This study compares the difference in outcomes between patients with AL receiving chemotherapy alone (CT) vs. AHCT. Seventy-four patients with AL were retrospectively analyzed. Two cohorts of patients were studied, those receiving CT (n=31) or AHCT (n=43)...
May 22, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28545410/combined-proximal-tubulopathy-crystal-storing-histiocytosis-and-cast-nephropathy-in-a-patient-with-light-chain-multiple-myeloma
#20
Chung-Kuan Wu, An-Hang Yang, Hung-Chih Lai, Bing-Shi Lin
BACKGROUND: The diagnosis of myeloma, a plasma dyscrasia, often results from the workup of unexplained renal disease. Persistent renal failure in myeloma is commonly caused by tubular nephropathy due to circulating immunoglobulins and free light chains. Myeloma cast nephropathy is characterized by crystalline precipitates of monoclonal light chains within distal tubules. Immunoglobulin crystallization rarely occurs intracellularly, within proximal tubular cells (light chain proximal tubulopathy) and interstitial histiocytes (crystal-storing histiocytosis)...
May 25, 2017: BMC Nephrology
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