keyword
MENU ▼
Read by QxMD icon Read
search

Light chain amyloidosis

keyword
https://www.readbyqxmd.com/read/28637305/left-atrial-structure-and-function-in-cardiac-amyloidosis
#1
Kotaro Nochioka, Candida Cristina Quarta, Brian Claggett, Gabriela Querejeta Roca, Claudio Rapezzi, Rodney H Falk, Scott D Solomon
Aims: Although cardiac amyloidosis (CA) is characterized by significant left atrial (LA) dilatation, the characteristics of LA function remain to be fully investigated. Methods and results: We assessed LA function by speckle-tracking echocardiography in 124 patients with CA and sinus rhythm: 68 with light chain (AL), 29 with mutant (ATTRm), 27 with wild-type (ATTRwt) transthyretin amyloidosis. Conventional and strain-derived parameters, including LA peak longitudinal strain (LS) and strain rate (peak LSR: reservoir function; early LSR: conduit function; late LSR: active function), were assessed compared between CA patients and 20 healthy controls of similar age and gender...
June 16, 2017: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/28636627/plasma-cell-neoplasia-after-kidney-transplantation-french-cohort-series-and-review-of-the-literature
#2
Raphaël Kormann, Hélène François, Thibault Moles, Jacques Dantal, Nassim Kamar, Karine Moreau, Thomas Bachelet, Anne-Elisabeth Heng, Antoine Garstka, Charlotte Colosio, Didier Ducloux, Johnny Sayegh, Benjamin Savenkoff, Denis Viglietti, Rebecca Sberro, Eric Rondeau, Julie Peltier
Although post-transplant lymphoproliferative disorder (PTLD) is the second most common type of cancer in kidney transplantation (KT), plasma cell neoplasia (PCN) occurs only rarely after KT, and little is known about its characteristics and evolution. We included twenty-two cases of post-transplant PCN occurring between 1991 and 2013. These included 12 symptomatic multiple myeloma, eight indolent myeloma and two plasmacytomas. The median age at diagnosis was 56.5 years and the median onset after transplantation was 66...
2017: PloS One
https://www.readbyqxmd.com/read/28632471/lenalidomide-as-a-treatment-for-relapsed-al-amyloidosis-in-an-hiv-positive-patient
#3
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/28632454/localized-amyloidogenic-immunoglobulin-light-chain-derived-amyloidosis-in-a-young-boy-and-an-adolescent-girl
#4
Shu-Ichi Ikeda
No abstract text is available yet for this article.
June 20, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
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
#5
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/28630445/myocardial-deformation-in-cardiac-amyloid-light-chain-amyloidosis-assessed-with-3t-cardiovascular-magnetic-resonance-feature-tracking
#6
Rui Li, Zhi-Gang Yang, Hua-Yan Xu, Ke Shi, Xi Liu, Kai-Yue Diao, Ying-Kun Guo
Clinically, assessment of myocardial function is essential in patients with amyloid light-chain cardiac amyloidosis (AL-CA) to predict outcome and determine therapeutic approach. The aim of this study was to investigate the feasibility of cardiovascular magnetic resonance (CMR)-derived feature tracking algorithm for assessing left ventricular (LV) myocardial deformation in AL-CA, and to determine if these abnormal myocardial deformation parameters are correlated to impaired LV myocardial microvascular dysfunction...
June 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28622303/addition-of-cyclophosphamide-and-higher-doses-of-dexamethasone-do-not-improve-outcomes-of-patients-with-al-amyloidosis-treated-with-bortezomib
#7
E Kastritis, M Gavriatopoulou, M Roussou, D Fotiou, D C Ziogas, M Migkou, E Eleutherakis-Papaiakovou, I Panagiotidis, N Kanellias, E Psimenou, E Papadopoulou, C Pamboucas, E Manios, H Gakiopoulou, A Ntalianis, A Tasidou, S Giannouli, E Terpos, M A Dimopoulos
Bortezomib, in combination with dexamethasone (VD) or with the addition of cyclophosphamide (VCD), is highly effective in patients with amyloid light-chain (AL) amyloidosis. Currently, VCD is considered as a primary regimen for patients with AL, but it is not clear whether the addition of cyclophosphamide to VD further and significantly improves efficacy, given the substantial activity of bortezomib itself. We retrospectively compared the outcomes of 101 patients with AL amyloidosis who received VD (n=59) or VCD (n=42) in two consecutive periods...
June 16, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/28616786/laryngotracheal-involvement-in-systemic-light-chain-amyloidosis
#8
Daniel T Ginat, Jefree Schulte, Louis Portugal, Nicole A Cipriani
Laryngotracheal amyloid deposition is an uncommon manifestation of systemic light chain amyloidosis. Diagnostic imaging, such as CT, is useful for suggesting the possibility of amyloidosis and delineating the extent of the lesions for surgical management; however, the diagnosis is confirmed with the histologic finding of amorphous eosinophilic material which stains positively for Congo red and may show apple green birefringence on polarization. These features are exemplified in this sine qua non radiology-pathology correlation article...
June 14, 2017: Head and Neck Pathology
https://www.readbyqxmd.com/read/28615223/daratumumab-yields-rapid-and-deep-hematologic-responses-in-patients-with-heavily-pretreated-al-amyloidosis
#9
Gregory P Kaufman, Stanley L Schrier, Richard A Lafayette, Sally Arai, Ronald M Witteles, Michaela Liedtke
The majority of patients with immunoglobulin light chain amyloidosis (AL) fail to achieve a complete response (CR) to standard light chain suppressive chemotherapy, and almost all patients eventually experience hematologic relapse and progression of organ involvement; additional well tolerated treatment options are therefore needed. We present our retrospective experience of 25 consecutive previously treated AL patients who received daratumumab, a CD38-directed monoclonal antibody approved for the treatment of multiple myeloma...
June 14, 2017: Blood
https://www.readbyqxmd.com/read/28613962/significant-association-between-renal-function-and-area-of-amyloid-deposition-in-kidney-biopsy-specimens-in-both-aa-amyloidosis-associated-with-rheumatoid-arthritis-and-al-amyloidosis
#10
Takeshi Kuroda, Naohito Tanabe, Eriko Hasegawa, Ayako Wakamatsu, Yukiko Nozawa, Hiroe Sato, Takeshi Nakatsue, Yoko Wada, Yumi Ito, Naofumi Imai, Mitsuhiro Ueno, Masaaki Nakano, Ichiei Narita
The kidney is a major target organ for systemic amyloidosis, which results in proteinuria and an elevated serum creatinine level. The clinical manifestations and precursor proteins of amyloid A (AA) and light-chain (AL) amyloidosis are different, and the renal damage due to amyloid deposition also seems to differ. The purpose of this study was to clarify haw the difference in clinical features between AA and AL amyloidosis are explained by the difference in the amount and distribution of amyloid deposition in the renal tissues...
June 14, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28611868/gastrointestinal-tract-amyloidosis-presenting-with-pneumatosis-intestinalis
#11
Vikram Raghunathan, David Louis, Baldeep Wirk
Pneumatosis intestinalis is a radiographic finding of gas pockets within the bowel wall. It can be associated with a range of diagnoses, but the most life-threatening causes are mesenteric ischemia, bowel necrosis, and bowel obstruction. Here we present the case of a patient with multiple myeloma who had pneumatosis intestinalis due to gastrointestinal amyloidosis, which is a rare manifestation of systemic amyloid disease. The patient had both transthyretin (ATTR) amyloidosis and acquired apolipoprotein serum amyloid A (AA) amyloidosis that are not usually seen in conjunction with multiple myeloma, which is most commonly associated with light-chain (AL) amyloidosis...
July 2017: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/28588817/nasopharyngeal-amyloidoma-with-osteolytic-lesions-leading-to-diagnosis-of-systemic-light-chain-amyloidosis
#12
Sandra Susanibar-Adaniya, Pankaj Mathur, Rodolfo Henrich Lobo, Daisy Alapat, Manoj Kumar, Christoph Heuck, Sharmilan Thanendrarajan
Amyloidomas of the head and neck region are uncommon and generally considered a benign localized form of amyloidosis. Here, we describe "the unusual case of a young man" with a nasopharyngeal mass and osteolytic lesions caused by systemic light-chain amyloidosis treated successfully with a combined surgical and chemotherapy approach.
June 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28579588/a-case-of-primary-amyloidosis-with-spontaneous-hepatic-rupture
#13
Tsuyoshi Suda, Hikaru Oguri
A 79-year-old man was diagnosed to have primary amyloid light-chain (AL) amyloidosis with associated liver damage and prominent hepatomegaly. He was followed up without any treatment. One year after the diagnosis, he was taken to the hospital with a sudden onset of features of shock. Computed tomography revealed hepatic rupture, and he was treated by emergent transcutaneous arterial embolization. However, the procedure was unable to save his life. AL amyloidosis with prominent hepatomegaly is considered to be a risk factor for spontaneous liver rupture and intra-abdominal hemorrhage...
2017: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
https://www.readbyqxmd.com/read/28579468/gallic-acid-induced-dose-dependent-inhibition-of-lysozyme-fibrillation
#14
Mouli Konar, Sudipta Bag, Pritam Roy, Swagata Dasgupta
Amyloidosis is primarily characterized by the deposition of misfolded protein aggregates. Although the natural polyphenols have long been known as effective amyloid inhibitors, the mechanistic details of their inhibitory actions still remain unclear. Our present study explores the inhibition mechanism of polyphenols by studying the anti-amyloidogenic property of gallic acid (GA), the smallest structural unit of tea polyphenols, on hen egg white lysozyme (HEWL) at physiological pH. Using various spectroscopic techniques such as UV-vis, fluorescence, circular dichroism and dynamic light scattering, and microscopic techniques such as TEM and FESEM, it has been shown that GA potentially inhibits the self-aggregation process in a concentration dependent manner...
June 1, 2017: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/28567484/measurement-of-liver-and-spleen-interstitial-volume-in-patients-with-systemic-amyloid-light-chain-amyloidosis-using-equilibrium-contrast-ct
#15
Jason Yeung, S Sivarajan, T A Treibel, S Rosmini, M Fontana, J D Gillmore, P N Hawkins, S Punwani, J C Moon, S A Taylor, S Bandula
OBJECTIVES: To investigate equilibrium contrast-enhanced CT (EQ-CT) measurement of extracellular volume fraction (ECV) in patients with systemic amyloid light-chain (AL) amyloidosis, testing the hypothesis that ECV becomes elevated in the liver and spleen and ECV correlates with other estimates of organ amyloid burden. METHODS: 26 patients with AL amyloidosis underwent EQ-CT, and ECV was measured in the liver and spleen. Patients also underwent serum amyloid P (SAP) component scintigraphy with grading of liver and spleen involvement...
May 31, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28560302/three-cases-of-localized-cutaneous-nodular-amyloidosis-in-patients-with-limited-systemic-sclerosis-and-a-brief-literature-review
#16
L S Goettsche, M S Moye, A J Tschetter, M S Stone, K A Wanat
Localized cutaneous nodular amyloidosis (LCNA) is the rarest form of localized cutaneous amyloidosis. In patients with LCNA, local plasma cells secrete immunoglobulin light chains called amyloid L via an unknown mechanism. LCNA has been associated with autoimmune connective tissue diseases such as most commonly Sjögren syndrome. A few reported cases of LCNA are associated with limited systemic sclerosis (LSSc). We report three cases of LCNA in patients with LSSc to add to the existing literature, discuss the disease association and proposed pathophysiology, and briefly review the existing information in current literature...
June 2017: International Journal of Women's Dermatology
https://www.readbyqxmd.com/read/28550043/al-amyloidosis-patients-with-low-amyloidogenic-free-light-chain-levels-at-first-diagnosis-have-an-excellent-prognosis
#17
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
#18
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
#19
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-amyloidosis
#20
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, Robert 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. In this study, we compared outcomes between patients with AL receiving chemotherapy alone (CT) and those undergoing AHCT. Seventy-four patients with AL were analyzed retrospectively. Two cohorts of patients were studied, those receiving CT (n = 31) and those undergoing AHCT (n = 43)...
May 22, 2017: Biology of Blood and Marrow Transplantation
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"