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Cardiac amyloidosis

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https://www.readbyqxmd.com/read/28647518/increasing-the-accuracy-of-proteomic-typing-by-decellularisation-of-amyloid-tissue-biopsies
#1
P Patrizia Mangione, Giuseppe Mazza, Janet A Gilbertson, Nigel B Rendell, Diana Canetti, Sofia Giorgetti, Luca Frenguelli, Marco Curti, Tamer Rezk, Sara Raimondi, Mark B Pepys, Philip N Hawkins, Julian D Gillmore, Graham W Taylor, Massimo Pinzani, Vittorio Bellotti
Diagnosis and treatment of systemic amyloidosis depend on accurate identification of the specific amyloid fibril protein forming the tissue deposits. Confirmation of monoclonal immunoglobulin light chain amyloidosis (AL), requiring cytotoxic chemotherapy, and avoidance of such treatment in non-AL amyloidosis, are particularly important. Proteomic analysis characterises amyloid proteins directly. It complements immunohistochemical staining of amyloid to identify fibril proteins and gene sequencing to identify mutations in the fibril precursors...
June 21, 2017: Journal of Proteomics
https://www.readbyqxmd.com/read/28646538/a-novel-attr-l32v-mutation-causes-familial-amyloid-polyneuropathy-in-a-bolivian-family
#2
P L Martínez-Ulloa, M Vallejo, I Corral, N García-Barragán, A Alcazar, Emma Martínez-Alonso, J Martínez-Poles, H Pian, A Jiménez-Escrig
We report a new transthyretin (ATTR) gene c.272C>G mutation and variant protein, p.Leu32Val, in a kindred of Bolivian origin with a rapid progressive peripheral neuropathy and cardiomyopathy. Three individuals from a kindred with peripheral nerve and cardiac amyloidosis were examined. Analysis of the TTR gene was performed by Sanger direct sequencing. Neuropathologic examination was obtained on the index patient with mass spectrometry study of the ATTR deposition. Direct DNA sequence analysis of exons 2, 3 and 4 of the TTR gene demonstrated a c...
June 24, 2017: Journal of the Peripheral Nervous System: JPNS
https://www.readbyqxmd.com/read/28639774/-discovery-of-left-ventricular-hypertrophy-during-adult-echocardiography
#3
Nicolas B Dayal, Hajo Müller
Left ventricular hypertrophy is a common finding during echocardiography. A precise evaluation of the left ventricular wall thickness, ventricular mass and distribution of hypertrophy is crucial both for diagnostic workup, follow-up and for prognostic evaluation. The differential diagnosis of left ventricular hypertrophy includes hypertrophic cardiomyopathies, hypertrophy secondary to abnormal left ventricular filling conditions, hypertrophy linked to intense physical training and the isolated basal septal hypertrophy of the elderly...
May 24, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28637305/left-atrial-structure-and-function-in-cardiac-amyloidosis
#4
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/28632471/lenalidomide-as-a-treatment-for-relapsed-al-amyloidosis-in-an-hiv-positive-patient
#5
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/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/28616564/regression-of-cardiac-amyloidosis-following-stem-cell-transplantation-a-comparison-between-echocardiography-and-cardiac-magnetic-resonance-imaging-in-long-term-survivors
#7
Benjamin Thomas Fitzgerald, John Bashford, Katrina Newbigin, Gregory Malcolm Scalia
BACKGROUND: AL amyloidosis and multiple myeloma result in extracellular deposition of insoluble fibrillary protein in tissue and organs. Untreated median survival is very poor, and even worse with cardiac involvement. Chemotherapy and peripheral blood stem cell transplantation (PBSCT) have been shown to dramatically improve survival, with hematologic remission documented. Regression of cardiac changes has previously been shown, as assessed by echocardiography (TTE) and cardiac magnetic resonance imaging (CMR)...
March 2017: IJC Heart & Vasculature
https://www.readbyqxmd.com/read/28615223/daratumumab-yields-rapid-and-deep-hematologic-responses-in-patients-with-heavily-pretreated-al-amyloidosis
#8
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/28611935/gastrointestinal-amyloidosis-review-of-the-literature
#9
REVIEW
Kyle Rowe, Jon Pankow, Fredy Nehme, William Salyers
Gastrointestinal amyloidosis (GIA), a protein deposition disorder, represents a complex common pathway that encompasses multiple etiologies and presentations. It represents a significant diagnostic and treatment challenge. The disease results from the deposition of insoluble extracellular protein fragments that have been rendered resistant to digestion. GIA can be acquired or genetic, and most commonly results from chronic inflammatory disorders (AA amyloidosis), hematologic malignancy (AL amyloidosis), and end-stage renal disease (Beta-2 amyloidosis)...
May 8, 2017: Curēus
https://www.readbyqxmd.com/read/28605421/diagnostic-sensitivity-of-abdominal-fat-aspiration-in-cardiac-amyloidosis
#10
Candida Cristina Quarta, Esther Gonzalez-Lopez, Janet A Gilbertson, Nichola Botcher, Dorota Rowczenio, Aviva Petrie, Tamer Rezk, Taryn Youngstein, Shameem Mahmood, Sajitha Sachchithanantham, Helen J Lachmann, Marianna Fontana, Carol J Whelan, Ashutosh D Wechalekar, Philip N Hawkins, Julian D Gillmore
Aims: Congo red staining of an endomyocardial biopsy is the diagnostic gold-standard in suspected cardiac amyloidosis (CA), but the procedure is associated with the risk, albeit small, of serious complications, and delay in diagnosis due to the requirement for technical expertise. In contrast, abdominal fat pad fine needle aspiration (FPFNA) is a simple, safe and well-established procedure in systemic amyloidosis, but its diagnostic sensitivity in patients with suspected CA remains unclear...
March 7, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28598686/how-your-ears-can-tell-what-is-hidden-in-your-heart-wild-type-transthyretin-amyloidosis-as-potential-cause-of-sensorineural-hearing-loss-inelderly-amylodeafness-pilot-study
#11
Emilie Béquignon, Aziz Guellich, Sophie Barthier, Marc Raynal, Virginie Prulière-Escabasse, Florence Canouï-Poitrine, André Coste, Thibaud Damy
BACKGROUND: Wild-type transthyretin amyloidosis (ATTRwt) is an age-related life-threatening condition. Prognosis is mainly dependent on cardiac involvement. Other organs and tissues may be affected. Their early recognition may increase awareness of physicians and positively affects the prognosis. Presbycusis is another age-related disorder. Whether this disease is associated to ATTRwt amyloidosis is unknown. METHODS: Sixteen consecutive patients with confirmed diagnosis of ATTRwt amyloidosis at the Mondor Amyloidosis Network, France, underwent otoscopy and audiological tests including pure tone audiometry, speech reception threshold and speech discrimination score...
June 9, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28588865/diffuse-large-b-cell-lymphoma-mimicking-cardiac-amyloidosis
#12
Lawrence Lau, Viktoriya Mozolevska, Iain D C Kirkpatrick, Davinder S Jassal, Roopesh Kansara
Our case highlights that cardiac lymphoma may mimic amyloid infiltration of the myocardium on cardiac magnetic resonance (CMR), and is a particularly challenging diagnosis in the setting of transformed Waldenström's macroglobulinemia. Heightened suspicion and early diagnosis of cardiac lymphoma is paramount as chemotherapy has been demonstrated to portent an increased survival rate.
June 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28585032/emerging-role-of-echocardiography-cardiac-magnetic-resonance-imaging-and-99m-tc-labeled-bone-tracer-scintigraphy-for-the-diagnosis-of-cardiac-amyloidosis
#13
EDITORIAL
Samia Massalha, Terrence D Ruddy
No abstract text is available yet for this article.
June 5, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28571197/cardiac-amyloidosis-an-infiltrative-heart-disease-presenting-as-arrhythmia-a-case-report
#14
B Magesh, Deepak Kadeli, Sunil Bohra, V Krishnaprasath, R Keshava
Cardiac amyloidosis is a manifestation of amyloidosis which is a multisystem disorder. This is difficult to diagnose, rare disease which eventually leads to the mortality. Diagnosis requires a high index of clinical suspicion along with echocardiographic clues like, diastolic dysfunction, bi-atrial enlargement and ventricular thickening. Treatment is mainly supportive with disappointing outcomes. We present a case of systemic amyloidosis with negative congo red staining, presenting with predominantly cardiac features...
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28553897/diagnostic-score-for-the-detection-of-cardiac-amyloidosis-in-patients-with-left-ventricular-hypertrophy-and-impact-on-prognosis
#15
Eve Cariou, Youssef Bennani Smires, Gérard Victor, Guillaume Robin, David Ribes, Pierre Pascal, Antoine Petermann, Pauline Fournier, Stanislas Faguer, Jérôme Roncalli, Hervé Rousseau, Dominique Chauveau, Didier Carrié, Isabelle Berry, Michel Galinier, Olivier Lairez
BACKGROUND: Among diagnosis associated with left ventricular hypertrophy (LVH), cardiac amyloidosis (CA) is a progressive disease with poor prognosis. Early noninvasive identification is of growing clinical importance. The objective of our study was to integrate clinical, biologic, electrocardiographic and echocardiographic parameters to build a diagnostic score in patients with LVH. METHODS AND RESULTS: One hundred and fourteen patients with LVH underwent a cardiac magnetic resonance (CMR) and a (99m)Tc-hydroxymethylene-diphosphonate scintigraphy ((99m)Tc-HMDP) allowing to discriminate three groups of diagnoses: CA (n = 50 including 31, 18 and 1 ATTR, AL and AA amyloidosis), hypertrophic cardiomyopathy (n = 19) and unspecific cardiomyopathy (n = 45)...
May 29, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
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/28537770/automated-description-of-regional-left-ventricular-motion-in-patients-with-cardiac-amyloidosis-a-quantitative-study-using-heart-deformation-analysis
#18
Leng Meng, Kai Lin, Jeremy Collins, Michael Markl, James C Carr
OBJECTIVE: The purpose of this article is to test the hypothesis that heart deformation analysis can automatically quantify regional myocardial motion patterns in patients with cardiac amyloidosis. SUBJECTS AND METHODS: Eleven patients with cardiac amyloidosis and 11 healthy control subjects were recruited to undergo cardiac MRI. Cine images were analyzed using heart deformation analysis and feature tracking. Heart deformation analysis-derived myocardial motion indexes in radial and circumferential directions, including radial and circumferential displacement, radial and circumferential velocity, radial and circumferential strain, and radial and circumferential strain rate, were compared between the two groups...
May 24, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28537040/accuracy-of-99mtc-hydroxymethylene-diphosphonate-scintigraphy-for-diagnosis-of-transthyretin-cardiac-amyloidosis
#19
Francesco Cappelli, Chiara Gallini, Carlo Di Mario, Egidio Natalino Costanzo, Luca Vaggelli, Francesca Tutino, Alfonso Ciaccio, Simone Bartolini, Paola Angelotti, Sabrina Frusconi, Silvia Farsetti, Giuseppe Vergaro, Assuero Giorgetti, Paolo Marzullo, Dario Genovesi, Michele Emdin, Federico Perfetto
BACKGROUND AND AIM: Either 99mTechnetium diphosphonate (Tc-DPD) or pyrophosphate (Tc-PYP) scintigraphy plays a relevant role in diagnosing transthyretin cardiac amyloidosis (CA), and labeled radiotracers have been extensively studied in diagnosing CA. Few studies have analyzed and validated 99mTc-Hydroxymethylene diphosphonate (Tc-HMDP). Our aim was to validate the diagnostic accuracy of Tc-HMDP total-body scintigraphy in a cohort of patients with biopsy-proven transthyretin CA. METHODS AND RESULTS: We retrospectively evaluated all patients undergoing 99mTc-HMDP total-body scintigraphy, in adjunct to a comprehensive diagnostic work-up for suspected CA...
May 23, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28536682/a-case-of-intramural-coronary-amyloidosis-associated-with-hemodialysis
#20
Faisal M Huq Ronny, George Kleinman, Paul James Kurtin, John Thomas Fallon
Dialysis-related amyloidosis predominantly occurs in osteo-articular structures and dialysis-related amyloid (DRA) substances also deposit in extra-articular tissues. Clinical manifestations of DRA include odynophagia, gastrointestinal hemorrhage, intestinal obstruction, kidney stones, myocardial dysfunction, and subcutaneous tumors. The pathological characteristics of DRA in the heart of hemodialysis patients have rarely been reported. We report the case of a 73-year-old female with a history of cerebral palsy and end-stage renal disease status post two failed renal transplants who had been on hemodialysis for 30 years...
January 2017: Autopsy & case reports
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