keyword
MENU ▼
Read by QxMD icon Read
search

Light chain amyloidosis

keyword
https://www.readbyqxmd.com/read/28522573/lenalidomide-melphalan-dexamethasone-in-newly-diagnosed-patients-with-al-amyloidosis-results-of-a-prospective-phase-2-study-with-long-term-follow-up
#1
Ute Hegenbart, Tilmann Bochtler, Axel Benner, Natalia Becker, Christoph Kimmich, Arnt V Kristen, Jörg Beimler, Ernst Hund, Markus Zorn, Anja Freiberger, Marianne Gawlik, Hartmut Goldschmidt, Dirk Hose, Anna Jauch, Anthony D Ho, Stefan O Schönland
Chemotherapy in light chain amyloidosis aims to normalize the involved free light chain in serum, which leads to an improvement or at least stabilization of organ function in most responding patients. We performed a prospective single center phase 2 trial with 50 untreated patients not eligible for high-dose treatment. The treatment schedule comprised 6 cycles of oral lenalidomide, melphalan and dexamethasone every 4 weeks. After 6 months complete remission was achieved in 9 patients (18%), very good partial remission in 16 (32%) and partial response in 9 (18%)...
May 18, 2017: Haematologica
https://www.readbyqxmd.com/read/28509352/convergent-mechanisms-favor-fast-amyloid-formation-in-two-lambda-6a-ig-light-chain-mutants
#2
Gilberto Valdés-García, Cesar Millan-Pacheco, Nina Pastor
Extracellular deposition as amyloids of immunoglobulin light chains causes light chain amyloidosis. Among the light chain families, lambda 6a is one of the most frequent in light chain amyloidosis patients. Its germline protein, 6aJL2, and point mutants, R24G and P7S, are good models to study fibrillogenesis, because their stability and fibril formation characteristics have been described. Both mutations make the germline protein unstable and speed up its ability to aggregate. To date, there is no molecular mechanism that explains how these differences in amyloidogenesis can arise from a single mutation...
May 16, 2017: Biopolymers
https://www.readbyqxmd.com/read/28508985/light-chain-fanconi-syndrome-in-a-patient-with-acute-myeloid-leukemia-and-monoclonal-gammopathy-of-undetermined-significance
#3
Daniel W Ross, Rimda Wanchoo, Adriana Guigova, Cristina Ghiuzeli, Steven L Allen, Kenar D Jhaveri
Proximal tubules are a target for paraproteinemic diseases. Cast nephropathy, light chain deposition diseases, and amyloidosis are frequently encountered in patients with multiple myeloma. Rarely, a subset of patients develop light chain Fanconi syndrome (LCFS). LCFS has been reported with multiple myeloma, monoclonal gammopathy of renal significance (MGRS), chronic lymphocytic leukemia, Waldenstrom's macroglobulinemia and diffuse large B-cell lymphoma. No cases have been described with other hematologic malignancies...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28508350/targeted-nuclear-imaging-probes-for-cardiac-amyloidosis
#4
REVIEW
Paco E Bravo, Sharmila Dorbala
PURPOSE OF REVIEW: The aim of the present manuscript is to review the latest advancements of radionuclide molecular imaging in the diagnosis and prognosis of individuals with cardiac amyloidosis. RECENT FINDINGS: (99m)Technetium labeled bone tracer scintigraphy had been known to image cardiac amyloidosis, since the 1980s; over the past decade, bone scintigraphy has been revived specifically to diagnose transthyretin cardiac amyloidosis. (18)F labeled and (11)C labeled amyloid binding radiotracers developed for imaging Alzheimer's disease, have been repurposed since 2013, to image light chain and transthyretin cardiac amyloidosis...
July 2017: Current Cardiology Reports
https://www.readbyqxmd.com/read/28505007/al-amyloidoma-of-the-skin-subcutis-cutaneous-amyloidosis-plasma-cell-dyscrasia-or-a-manifestation-of-primary-cutaneous-marginal-zone-lymphoma
#5
Noreen M Walsh, Ian Marie Lano, Peter Green, Christopher Gallant, Sylvia Pasternak, Thai Yen Ly, Luis Requena, Heinz Kutzner, Andreas Chott, Lorenzo Cerroni
It is unclear whether AL amyloidoma of the skin/subcutis represents a distinct entity, an indolent precursor of systemic amyloidosis, or a manifestation of cutaneous marginal zone lymphoma (cMZL). We collected 10 cases of cutaneous AL amyloidoma in order to better characterize the clinicopathologic features of this elusive entity (M:F=4:6; median age: 62.5 y, range: 31 to 82 y). Nine patients had a solitary nodule or plaque on the lower extremity (n=7), upper extremity (n=1), or chin (n=1). One patient had an AL amyloidoma on the right thigh and a second lesion on the right arm showing histopathologic features of cMZL without amyloid deposits...
May 12, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28490298/raccoon-eyes-in-systemic-light-chain-amyloidosis
#6
Cecily J Forsyth, Campbell R Tiley
No abstract text is available yet for this article.
May 15, 2017: Medical Journal of Australia
https://www.readbyqxmd.com/read/28483281/preclinical-data-support-leveraging-cs1-chimeric-antigen-receptor-t-cell-therapy-for-systemic-light-chain-amyloidosis
#7
Michael Rosenzweig, Ryan Urak, Miriam Walter, Laura Lim, James F Sanchez, Amrita Krishnan, Stephen Forman, Xiuli Wang
BACKGROUND AIMS: Light chain amyloidosis (AL) is a protein deposition disorder that is a result of a plasma cell dyscrasia, similar to multiple myeloma (MM). Immunotherapy is an attractive approach because of the low burden of disease, but the optimal target for AL is unclear. CS1 and B-cell maturation antigen (BCMA) are two potential targets because they are expressed on normal plasma cells and MM cells. METHODS: We performed a prospective study evaluating bone marrow specimens of 20 patients with plasma cell diseases, 10 with AL and 10 with MM...
May 5, 2017: Cytotherapy
https://www.readbyqxmd.com/read/28483059/presentation-and-outcomes-of-localized-immunoglobulin-light-chain-amyloidosis-the-mayo-clinic-experience
#8
Taxiarchis V Kourelis, Robert A Kyle, David Dingli, Francis K Buadi, Shaji K Kumar, Morie A Gertz, Martha Q Lacy, Prashant Kapoor, Ronald S Go, Wilson I Gonsalves, Rahma Warsame, John A Lust, Suzanne R Hayman, S Vincent Rajkumar, Steven R Zeldenrust, Stephen J Russell, Yi Lin, Nelson Leung, Angela Dispenzieri
OBJECTIVE: To describe treatment types, outcomes, and relapse patterns in patients with localized immunoglobulin light chain amyloidosis (ALL). PATIENTS AND METHODS: We included all patients with ALL seen at Mayo Clinic in Rochester, Minnesota, from January 1, 1968, through June 30, 2014. The diagnosis of ALL was predicated on the presence of a Congo red-positive biopsy specimen and negative serum and urine immunofixation. Treatment response categories were response, stability, and progression...
May 5, 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28466795/-amyloidosis-despite-normal-esr
#9
S M Kuipers, L de Groot, T R Zijlstra, N C Köster, A E van der Bijl
BACKGROUND: Amyloidosis is a clinical picture brought on by extracellular deposits of insoluble, non-degradable proteins. The clinical presentation of amyloidosis depends upon the type of protein and the organ afflicted. CASE DESCRIPTION: A 65-year-old woman had stiffness of the locomotor apparatus, recurrent carpal tunnel syndrome and problems with swallowing. Laboratory and urine investigations initially showed no abnormalities, but histopathological investigation of a synovial biopsy revealed amyloidosis with light-chain deposits...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28457657/stem-cell-transplantation-for-immunoglobulin-light-chain-amyloidosis
#10
REVIEW
Taimur Sher, Morie A Gertz
Systemic chemotherapy aimed at eradicating transformed plasma cells is the mainstay of treatment for immunoglobulin light chain amyloidosis (AL). Autologous stem cell transplantation (SCT) is a highly effective treatment for AL and can lead to long term survival in excess of 10 years in patients who achieve complete remission. Since AL is a unique disease characterized by multiple organ dysfunction, SCT poses unique challenges in this disease. Morbidity and mortality of SCT has remarkably improved over time primarily due to careful selection of patients and evolution of predictive and prognostic models based on serum immunoglobulin light chains and cardiac biomarkers...
March 16, 2017: Current Problems in Cancer
https://www.readbyqxmd.com/read/28456755/light-chain-cardiac-amyloidosis-strategies-to-promote-early-diagnosis-and-cardiac-response
#11
REVIEW
Martha Grogan, Angela Dispenzieri, Morie A Gertz
Amyloid light chain (AL) amyloidosis is a systemic disease characterised by the aggregation of misfolded immunoglobulin light chain (LC), predominantly in the heart and kidneys, causing organ failure. If untreated, the median survival of patients with cardiac AL amyloidosis is 6 months from the onset of heart failure. Protracted time to establish a diagnosis, often lasting >1 year, is a frequent factor in poor treatment outcomes. Cardiologists, to whom patients are often referred, frequently miss the opportunity to diagnose cardiac AL amyloidosis...
April 29, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28454452/flow-cytometry-based-immunophenotypic-analysis-of-primary-systemic-light-chain-amyloidosis
#12
Xiangwen Diao, Juan Li, Juan Ouyang, Junru Liu, Beihui Huang, Meilan Chen, Jingli Gu
Using flow cytometry, the present study aimed to investigate the immunophenotypic characteristics of malignant plasma cells (PCs) in the bone marrow of patients with primary systemic light chain amyloidosis (AL) compared with the characteristics of patients with multiple myeloma (MM). Flow cytometric results of 51 patients with AL and 150 patients with MM were reviewed. The proportion of total bone marrow PCs in the patients with AL was significantly lower than that in the patients with MM, 1.35% (0.3-9.5%) vs...
April 2017: Oncology Letters
https://www.readbyqxmd.com/read/28439985/the-utility-of-mass-fix-to-detect-and-monitor-monoclonal-proteins-in-the-clinic
#13
Paolo Milani, David L Murray, David R Barnidge, Mindy C Kohlhagen, John R Mills, Giampaolo Merlini, Surendra Dasari, Angela Dispenzieri
The detection and quantification of monoclonal-proteins (M-proteins) are necessary for the diagnosis and evaluation of response in plasma cell dyscrasias. Immunoglobulin enrichment-coupled with matrix-assisted laser desorption ionization time-of-flight mass-spectrometry (MASS-FIX) is a simple and inexpensive method to identify M-proteins, but its clinical generalizability has not yet been elucidated. We compared MASS-FIX to protein electrophoresis (PEL), serum/urine immunofixation-electrophoresis (IFE) and quantitative serum free-light chain (FLC) for the identification of M-proteins in different clinical diagnoses...
April 25, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28439924/prevalence-and-predictors-of-thyroid-functional-abnormalities-in-newly-diagnosed-al-amyloidosis
#14
E Muchtar, D S Dean, A Dispenzieri, D Dingli, F K Buadi, M Q Lacy, S R Hayman, P Kapoor, N Leung, S Russell, J A Lust, Yi Lin, R Warsame, W Gonsalves, T V Kourelis, R S Go, R Chakraborty, S Zeldenrust, R A Kyle, S Vincent Rajkumar, S K Kumar, M A Gertz
BACKGROUND: Data on the effect of systemic immunoglobulin light chain amyloidosis (AL amyloidosis) on thyroid function are limited. OBJECTIVE: To assess the prevalence of hypothyroidism in AL amyloidosis patients and determine its predictors. METHODS: 1142 newly diagnosed AL amyloidosis patients were grouped based on the thyroid-stimulating hormone (TSH) measurement at diagnosis: hypothyroid group (TSH above upper normal reference; >5 mIU L(-1) ; n = 217, 19% of study participants) and euthyroid group (n = 925, 81%)...
April 24, 2017: Journal of Internal Medicine
https://www.readbyqxmd.com/read/28434927/impact-of-autologous-stem-cell-transplantation-on-the-incidence-and-outcome-of-oligoclonal-bands-in-patients-with-light-chain-amyloidosis
#15
Luis Gerardo Rodríguez-Lobato, Carlos Fernández de Larrea, M Teresa Cibeira, Natalia Tovar, Juan I Aróstegui, Laura Rosiñol, Tania Díaz, Ester Lozano, Montserrat Elena, Jordi Yagüe, Joan Bladé
The emergence of oligoclonal bands (OB) in patients with multiple myeloma achieving a complete remission (CR) after autologous stem cell transplantation (ASCT) and the use of novel agents is a well-recognized event. The presence of OB is associated with favorable outcome. However, the emergence of OB in light-chain (AL) amyloidosis has never been investigated. The aim of the study was to determine the incidence, natural history, and prognostic impact of OB in 50 patients with AL amyloidosis who achieved at least a partial response either after upfront ASCT (20 patients [40%]) or after conventional treatment in patients ineligible for transplantation (30 patients [60%])...
April 18, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28434368/outcome-of-very-young-%C3%A2-40-years-patients-with-immunoglobulin-light-chain-al-amyloidosis
#16
Jithma P Abeykoon, Jonas Paludo, Angela Dispenzieri, Morie A Gertz, David Dingli, Francis K Baudi, Wilson I Gonsalves, Robert A Kyle, Martha Q Lacy, Suzanne R Hayman, Nelson Leung, Taxiarchis Kourelis, S Vincent Rajkumar, Shaji Kumar, Prashant Kapoor
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434343/patients-with-al-amyloidosis-and-low-free-light-chain-burden-have-distinct-clinical-features-and-outcome
#17
Paolo Milani, Marco Basset, Francesca Russo, Andrea Foli, Francesca Lavatelli, Mario Nuvolone, Giovanni Ferraro, Giovanni Palladini, Giampaolo Merlini
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434321/renal-outcomes-of-autologous-stem-cell-transplantation-among-patients-with-light-chain-amyloidosis-a-single-centre-spanish-experience
#18
Sebastián Ernesto Azorín, María Teresa Cibeira, Manel Solé, Carlos Fernández de Larrea, Laura Rosiñol, Montse Rovira, Josep Maria Campistol, Joan Bladé
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434310/diagnostic-accuracy-of-new-indirect-elisas-for-free-light-chain-quantification-in-fat-tissue-of-patients-with-al-amyloidosis
#19
Elisabeth Eelsing, Johan Bijzet, Bouke Hazenberg
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434299/first-report-of-myd88-l265p-somatic-mutation-in-igm-associated-light-chain-amyloidosis
#20
Rajshekar Chakraborty, Anne J Novak, Stepen M Ansell, Eli Muchtar, Prashant Kapoor, Suzanne R Hayman, Angela Dispenzieri, Francis K Buadi, Martha Q Lacy, Rebecca L King, Morie A Gertz
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
keyword
keyword
18746
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"