955 results match your criteria Light-Chain Deposition Disease


Multidisciplinary approach in the management of hATTR.

Eur J Clin Invest 2020 May 31:e13296. Epub 2020 May 31.

Internal Medicine Service. Hospital, Universitario Son Llàtzer, Crta Manacor Km 4. 07198, Palma, Balearic Islands, Spain.

Amyloidosis is a disease with various clinical presentations characterized by deposition of insoluble, misassembled fibril proteins (amyloid) that disrupt normal tissue structure and function. Amyloidosis is caused by up to 30 different proteins, including immunoglobulin light chain and transthyretin (TTR). Read More

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http://dx.doi.org/10.1111/eci.13296DOI Listing

Venetoclax in a Patient With Light Chain Deposition Disease Secondary to MGRS That Progressed After Kidney Transplantation.

Clin Lymphoma Myeloma Leuk 2020 Apr 7. Epub 2020 Apr 7.

Division of Hematology, Mayo Clinic, Rochester, MN.

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http://dx.doi.org/10.1016/j.clml.2020.03.013DOI Listing

Neuropathological and biochemical investigation of Hereditary Ferritinopathycases with ferritin light chain mutation: Prominent protein aggregation in the absence of major mitochondrial or oxidative stress.

Neuropathol Appl Neurobiol 2020 May 28. Epub 2020 May 28.

Wolfson Building, Newcastle University, Claremont Place, Newcastle upon Tyne, NE2 4AA, UK.

Aims: Neuroferritinopathyor Hereditary Ferritinopathy (HF)is an autosomal dominant movement disorder due to mutation in the light chain of the iron storage protein ferritin (FTL).HF is the only late-onset neurodegeneration with brain iron accumulation disorder andstudy of HF offers a unique opportunity to understand the role of iron in more common neurodegenerative syndromes.

Methods: We carried out pathological and biochemical studies of six individuals with the same pathogenic FTL mutation. Read More

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http://dx.doi.org/10.1111/nan.12634DOI Listing

IgM monoclonal gammopathy with heavy-and-light-chain amyloidosis resembling fibrillary glomerulonephritis determined by tandem mass spectrometry: a case report.

BMC Nephrol 2020 May 24;21(1):195. Epub 2020 May 24.

Department of Nephrology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.

Background: Fibrillary glomerulonephritis (FGN) is distinguished from amyloidosis by thicker fibrils and the lack of staining with histochemical dyes typically reactive with amyloid. However, congophilic FGN has been proposed recently and adding laser microdissection followed by mass spectrometry (LMD/MS) to conventional pathological methods would be helpful to diagnose FGN. Here, we report a patient initially diagnosed with FGN whose final pathological diagnosis was changed to immunoglobulin heavy-and-light-chain amyloidosis (AHL) after LMD/MS. Read More

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http://dx.doi.org/10.1186/s12882-020-01851-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7245905PMC

Renal amyloidosis: an update on diagnosis and pathogenesis.

Protoplasma 2020 May 24. Epub 2020 May 24.

Department of Biotechnology, School of Chemical and Life Sciences, Jamia Hamdard, New Delhi, 110062, India.

Amyloidosis is a diverse group of protein conformational disorder which is caused by accumulation and deposition of insoluble protein fibrils in vital tissues or organs, instigating organ dysfunction. Renal amyloidosis is characterized by the acellular Congo red-positive pathologic deposition of amyloid fibrils within glomeruli and/or the interstitium. It is generally composed of serum amyloid A-related protein or an immunoglobulin light chain; other rare forms lysozyme, gelsolin, fibrinogen alpha chain, transthyretin, apolipoproteins AI/AII/AIV/CII/CIII; and the recently identified form ALECT2. Read More

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http://dx.doi.org/10.1007/s00709-020-01513-0DOI Listing
May 2020
2.651 Impact Factor

Improved Quantification of Cardiac Amyloid Burden in Systemic Light Chain Amyloidosis: Redefining Early Disease?

JACC Cardiovasc Imaging 2020 May 8. Epub 2020 May 8.

Department of Medicine, Division of Cardiology, Cardiac Amyloidosis Program, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts; Department of Radiology, Division of Nuclear Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts; Department of Medicine and Radiology, CV Imaging Program, Cardiovascular Division, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts. Electronic address:

Objectives: The purpose of this study was to determine phenotypes characterizing cardiac involvement in AL amyloidosis by using direct (fluorine-18-labeled florbetapir {[F]florbetapir} positron emission tomography [PET]/computed tomography) and indirect (echocardiography and cardiac magnetic resonance [CMR]) imaging biomarkers of AL amyloidosis.

Background: Cardiac involvement in systemic light chain amyloidosis (AL) is the main determinant of prognosis and, therefore, guides management. The hypothesis of this study was that myocardial AL deposits and expansion of extracellular volume (ECV) could be identified before increases in N-terminal pro-B-type natriuretic peptide or wall thickness. Read More

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http://dx.doi.org/10.1016/j.jcmg.2020.02.025DOI Listing

Melatonin Attenuates Calcium Deposition from Vascular Smooth Muscle Cells by Activating Mitochondrial Fusion and Mitophagy via an AMPK/OPA1 Signaling Pathway.

Oxid Med Cell Longev 2020 24;2020:5298483. Epub 2020 Apr 24.

Department of Cardiology, Nanlou Division, Chinese PLA General Hospital at Beijing; National Clinical Research Center for Geriatric Diseases, China.

Mitochondrial fusion/mitophagy plays a role in cardiovascular calcification. Melatonin has been shown to protect against cardiovascular disease. This study sought to explore whether melatonin attenuates vascular calcification by regulating mitochondrial fusion/mitophagy via the AMP-activated protein kinase/optic atrophy 1 (AMPK/OPA1) signaling pathway. Read More

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http://dx.doi.org/10.1155/2020/5298483DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7196154PMC

Diagnosing cardiac amyloidosis in every-day practice: A practical guide for the cardiologist.

Int J Cardiol Heart Vasc 2020 Jun 27;28:100519. Epub 2020 Apr 27.

Department of Cardiology and Vascular Medicine, West German Heart and Vascular Center, University Hospital Essen, Hufelandstrasse 55, 45147 Essen, Germany.

Cardiac amyloidosis (CA) has emerged as a previously underestimated cause of heart failure and mortality. Underdiagnosis resulted mainly from unawareness of the true disease prevalence and the non-specific symptoms of the disease. CA results from extracellular deposition of misfolded protein fibrils, commonly derived from transthyretin (ATTR) or immunoglobulin light chains (AL). Read More

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http://dx.doi.org/10.1016/j.ijcha.2020.100519DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7191222PMC

Liver failure due to relapsed myeloma and hepatic iron overload.

Ecancermedicalscience 2020 30;14:1028. Epub 2020 Apr 30.

Department of Pathology, Cleveland Clinic Florida, Weston, Florida, USA.

Multiple myeloma is a hematologic malignancy that classically manifests with hypercalcaemia, renal insufficiency, anaemia and lytic bone lesions. Liver dysfunction in multiple myeloma is a lesser known complication that occurs through biliary obstruction, liver infiltration by plasma cells, amyloid/light chain deposition or due to liver injury from medications. Although transfusion-related hepatic iron overload-leading to significant liver disease-is a recognised complication in certain hematologic malignancies, little is known about transfusional iron overload in patients with multiple myeloma. Read More

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http://dx.doi.org/10.3332/ecancer.2020.1028DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7192399PMC

Mesenteric Amyloidosis: Radiologic Imaging with Pathologic Correlation.

J Clin Imaging Sci 2020 2;10:24. Epub 2020 May 2.

Departments of Radiology and Biomedical Imaging, Yale School of Medicine, Yale University, New Haven, Connecticut, United States.

Amyloidosis is a rare disease that is characterized by abnormal deposition of amyloid proteins in tissues, resulting in local, or systemic disease. When localized, it can present as an amyloidoma. We report a case of mesenteric amyloidosis in an 80-year-old male who was found to have an incidental mesenteric mass that was biopsy-proven to represent non-light chain amyloid tissue. Read More

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http://dx.doi.org/10.25259/JCIS_10_2020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7193195PMC

When to Suspect a Diagnosis of Amyloidosis.

Acta Haematol 2020 Apr 27:1-8. Epub 2020 Apr 27.

Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA,

Amyloidosis is a group of complex diseases caused by extracellular deposition of pathological insoluble fibrillary protein in organs and tissues and may result in severe organ dysfunction. Despite the etiological heterogeneity of systemic amyloidosis, the clinical manifestations of the different forms of amyloidosis largely overlap and depend upon the effected organ. The signs and symptoms that should raise suspicion for the potential diagnosis of amyloidosis are usually nonspecific; therefore, establishing the diagnosis is difficult, and early diagnosis requires clinical suspicion. Read More

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http://dx.doi.org/10.1159/000506617DOI Listing

Multiple morphological phenotypes of monoclonal immunoglobulin disease on renal biopsy: Significance of treatment.

Clin Nephrol Case Stud 2020 17;8:17-24. Epub 2020 Apr 17.

Department of Pathology, Cedars-Sinai Medical Center, Los Angeles, CA, USA.

Plasma cell dyscrasias frequently involve the kidney causing renal dysfunction. Multiple morphologic manifestations of κ light chain disease occurring simultaneously in the same kidney biopsy are uncommon and suggest local microenvironment effects in addition to structural properties of the light chain. A 61-year-old female presented with new onset renal failure and proteinuria. Read More

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http://dx.doi.org/10.5414/CNCS110052DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7171697PMC

A case report of pulmonary amyloidosis recognized by detection of AA amyloid exclusively in alveolar macrophages.

Respir Med Case Rep 2020 8;30:101046. Epub 2020 Apr 8.

Department of Pathology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.

Amyloidosis is a rare condition in which tissue deposits of inert fibrillar protein result in organ damage and dysfunction. There are several types of amyloid fibrils. Some of the most common forms are AL (amyloid light chain) protein and AA (amyloid-associated) type of amyloid fibril protein. Read More

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http://dx.doi.org/10.1016/j.rmcr.2020.101046DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7155221PMC

Lacrimal Gland Amyloidosis in an Elderly Patient.

Case Rep Ophthalmol 2020 Jan-Apr;11(1):100-105. Epub 2020 Mar 6.

Department of Ophthalmology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, Toyama, Japan.

Localized amyloidosis of the lacrimal gland is a rare disease. We report a case of transthyretin-positive localized amyloidosis of the lacrimal gland in a 74-year-old man with left lacrimal gland swelling. Biopsy of the left lacrimal gland showed extensive deposition of nonstructural eosinophilic material in the secretory gland and ducts, which stained positive with direct fast scarlet. Read More

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http://dx.doi.org/10.1159/000505480DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7154250PMC

Management of complications of cardiac amyloidosis: 10 questions and answers.

Eur J Prev Cardiol 2020 Apr 16:2047487320920756. Epub 2020 Apr 16.

Institute of Life Sciences, Scuola Superiore Sant'Anna, Italy.

Amyloidosis is a systemic disorder characterized by extracellular deposition of insoluble fibrils. The most common forms are amyloid light chain and amyloid transthyretin (ATTR) amyloidoses. Cardiac involvement may be found in both these forms, and is an important cause of morbidity and mortality. Read More

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http://dx.doi.org/10.1177/2047487320920756DOI Listing

Antibody-based immunotherapy for treatment of immunoglobulin light-chain amyloidosis.

Br J Haematol 2020 Apr 16. Epub 2020 Apr 16.

Department of Medicine, Hematology-Oncology, Mayo Clinic Florida, Jacksonville, FL, USA.

Immunoglobulin light-chain (AL) amyloidosis is a clonal plasma cell disorder characterised by production and deposition of misfolded monoclonal light chains in vital organs with potential to cause irreversible organ damage. The treatment of AL amyloidosis has evolved along the lines of multiple myeloma (MM) owing to clonal plasma cells being at the root of both disease processes. Treatment with melphalan and autologous haematopoietic cell transplantation, as well as proteasome inhibitors and immunomodulatory agents, are the standard of care for AL amyloidosis. Read More

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http://dx.doi.org/10.1111/bjh.16697DOI Listing

Amyloidosis-the Diagnosis and Treatment of an Underdiagnosed Disease.

Dtsch Arztebl Int 2020 Mar;117(10):159-166

Interdisciplinary Amyloidosis Center of Northern Bavaria, University Hospital Würzburg, Germany; Medical Clinic and Policlinic II, Dept. of Hemtatology, University Hospital Würzburg, Germany; Comprehensive Heart Failure Center Würzburg, University and University Hospital Würzburg, Germany; Medical Clinic and Policlinic I, Dept. of Cardiology, University Hospital Würzburg, Germany; Department of Neurology, University Hospital Würzburg, Germany; Medical Clinic and Policlinic II, Dept. of Hepatology, University Hospital Würzburg, Germany.

Background: Systemic amyloidosis is a multi-system disease caused by fibrillary protein deposition with ensuing dysfunction of the affected organ systems. Its diagnosis is often delayed because the manifestations of the disease are variable and non-specific. Its main forms are light chain (AL) amyloidosis and transthyretinrelated ATTR amyloidosis, which, in turn, has both a sporadic subtype (wildtype, ATTRwt) and a hereditary subtype (mutated, ATTRv). Read More

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http://dx.doi.org/10.3238/arztebl.2020.0159DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7171477PMC

Site-Specific Interactions with Copper Promote Amyloid Fibril Formation for λ6aJL2-R24G.

ACS Omega 2020 Apr 23;5(13):7085-7095. Epub 2020 Mar 23.

Centro de Investigación en Dinámica Celular-IICBA, Universidad Autónoma del Estado de Morelos, Cuernavaca, Morelos 62209, Mexico.

Light-chain amyloidosis (AL) is one of the most common systemic amyloidoses, and it is characterized by the deposition of immunoglobulin light chain (LC) variable domains as insoluble amyloid fibers in vital organs and tissues. The recombinant protein 6aJL2-R24G contains λ6a and JL2 germline genes and also contains the Arg24 by Gly substitution. This mutation is present in 25% of all amyloid-associated λ6 LC cases, reduces protein stability, and increases the propensity to form amyloid fibers. Read More

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http://dx.doi.org/10.1021/acsomega.9b03220DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7143407PMC

Amyloid in the Lung.

Semin Respir Crit Care Med 2020 Apr 12;41(2):299-310. Epub 2020 Apr 12.

Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, Minnesota.

Amyloidosis is the term given to abnormal deposition of misfolded precursor proteins at single or multiple sites, leading to organ dysfunction or clinical signs and symptoms. Pulmonary manifestations are nonspecific and may be associated with several amyloid protein subtypes, commonly AL (light chain) and AA (autoimmune) amyloids. Signs or symptoms of amyloid disease may often involve more of the clinical abnormalities of other affected organs than the lungs themselves. Read More

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http://dx.doi.org/10.1055/s-0040-1708059DOI Listing

Immunoglobulin light chain amyloidosis: 2020 update on diagnosis, prognosis, and treatment.

Authors:
Morie A Gertz

Am J Hematol 2020 Apr 8. Epub 2020 Apr 8.

Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.

Disease Overview: Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend on organs involved but can include heart failure with preserved ejection fraction, nephrotic syndrome, hepatic dysfunction, peripheral/autonomic neuropathy, and "atypical smoldering multiple myeloma or monoclonal gammopathy undetermined significance (MGUS)."

Diagnosis: Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple-green birefringence is required for diagnosis. Read More

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http://dx.doi.org/10.1002/ajh.25819DOI Listing

Heavy Lifting: Nomenclature and Novel Therapy for Gamma Heavy Chain Disease and Other Heavy Chain Disorders.

Clin Lymphoma Myeloma Leuk 2020 Mar 6. Epub 2020 Mar 6.

Division of Hematology, Department of Internal Medicine, The Ohio State University, Columbus, OH.

Heavy chain disorders are rare B-cell disorders and include heavy chain disease, heavy chain deposition disease, and heavy chain amyloidosis. These disorders share the pathognomonic finding of a truncated immunoglobulin heavy chain without an associated light chain in the serum or urine in the case of heavy chain disease or in the tissues in the case of heavy chain deposition disease and heavy chain amyloidosis but are clinically distinct entities. The clinical recognition and systematic approaches to these disorders are challenging because of the rarity of the diseases, lack of consensus on treatment approaches, and minimal data with novel therapy. Read More

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http://dx.doi.org/10.1016/j.clml.2020.02.020DOI Listing

Supportive Care in AL Amyloidosis.

Acta Haematol 2020 Apr 1:1-8. Epub 2020 Apr 1.

Hematology Department, Amyloidosis and Myeloma Unit, Hospital Clínic of Barcelona, University of Barcelona, IDIBAPS, Barcelona, Spain.

Immunoglobulin light-chain (AL) amyloidosis is a systemic disease characterized by the production and deposition of light chain-derived amyloid fibrils in different organs. Prompt treatment directed to the underlying plasma cell clone is crucial in order to achieve a rapid, deep and durable hematologic response. The decrease in the production of the amyloidogenic light chains is a required condition to obtain the organ response, which is commonly delayed. Read More

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http://dx.doi.org/10.1159/000506760DOI Listing

Pulmonary nodular and cystic light chain deposition disease: A retrospective review of 10 cases.

Respir Med 2020 Apr 14;164:105896. Epub 2020 Feb 14.

Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.

Introduction: Light chain deposition disease (LCDD) rarely involves the lungs. We report clinical and radiologic findings of pulmonary LCDD.

Methods: We retrospectively identified patients with biopsy-proven pulmonary LCDD seen at Mayo Clinic (Rochester, Minnesota) from January 1997 through December 2018. Read More

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http://dx.doi.org/10.1016/j.rmed.2020.105896DOI Listing

An update to the pathogenesis for monoclonal gammopathy of renal significance.

Crit Rev Oncol Hematol 2020 May 2;149:102926. Epub 2020 Mar 2.

Department of Nephrology, the Second Affiliated Hospital of Nanchang University, Nanchang, China. Electronic address:

Monoclonal gammopathy of renal significance (MGRS) is characterized by the nephrotoxic monoclonal immunoglobulin (MIg) secreted by an otherwise asymptomatic or indolent B-cell or plasma cell clone, without hematologic criteria for treatment. The spectrum of MGRS-associated disorders is wide, including non-organized deposits or inclusions such as C3 glomerulopathy with monoclonal glomerulopathy (MIg-C3G), monoclonal immunoglobulin deposition disease, proliferative glomerulonephritis with monoclonal immunoglobulin deposits and organized deposits like immunoglobulin related amyloidosis, type I and type II cryoglobulinaemic glomerulonephritis, light chain proximal tubulopathy, and so on. Kidney biopsy should be conducted to identify the exact disease associated with MGRS. Read More

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http://dx.doi.org/10.1016/j.critrevonc.2020.102926DOI Listing

Mitral and tricuspid stenosis caused by light chain cardiac amyloid deposition.

ESC Heart Fail 2020 Jun 16;7(3):1130-1135. Epub 2020 Mar 16.

Department of Cardiovascular Medicine, Cleveland Clinic, Cleveland, OH, 44195, USA.

Cardiac amyloidosis results in an infiltrative restrictive cardiomyopathy, with a number of characteristic features: biventricular hypertrophy, abnormal myocardial global longitudinal strain with relative apical sparing, biatrial dilation, and small pericardial effusion along with conduction abnormalities. Amyloid deposits leading to hemodynamically significant valvular heart disease are very rare. We describe a rare case of concomitant moderately severe tricuspid and mitral valve stenosis because of ongoing amyloid deposition in a patient with progressive multiple myeloma and fat pad biopsy-proven light chain amyloidosis. Read More

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http://dx.doi.org/10.1002/ehf2.12668DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7261535PMC

Amyloidosis as a Systemic Disease in Context.

Can J Cardiol 2020 Mar 17;36(3):396-407. Epub 2020 Jan 17.

Brigham and Women's Hospital Amyloidosis Program, Division of Cardiovascular Medicine Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

The systemic amyloidoses are a group of diseases characterized by the deposition of amyloid, a material formed from misfolding of proteins, in one or more organs. The 2 commonest forms of amyloidosis are transthyretin amyloidosis (ATTR), derived from wild-type or mutant transthyretin, and light-chain (AL) amyloidosis, derived from abnormal circulating light chains produced by plasma cell dyscrasia. Both frequently involve the heart, producing an infiltrative cardiomyopathy with restrictive pathophysiology. Read More

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http://dx.doi.org/10.1016/j.cjca.2019.12.033DOI Listing

Canadian Cardiovascular Society/Canadian Heart Failure Society Joint Position Statement on the Evaluation and Management of Patients With Cardiac Amyloidosis.

Can J Cardiol 2020 Mar;36(3):322-334

University of Manitoba, Winnipeg, Manitoba, Canada.

Cardiac amyloidosis is an under-recognized and potentially fatal cause of heart failure and other cardiovascular manifestations. It is caused by deposition of misfolded precursor proteins as fibrillary amyloid deposits in cardiac tissues. The two primary subtypes of systemic amyloidosis causing cardiac involvement are immunoglobulin light chain (AL), a plasma cell dyscrasia, and transthyretin (ATTR), itself subdivided into a hereditary subtype caused by a gene mutation of the ATTR protein, and an age-related wild type, which occurs in the absence of a gene mutation. Read More

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http://dx.doi.org/10.1016/j.cjca.2019.12.034DOI Listing
March 2020
3.711 Impact Factor

Clinical outcomes and prognostic factors in patients with multiple myeloma in South Tyrol: a retrospective single-center analysis.

Ann Hematol 2020 May 5;99(5):1031-1040. Epub 2020 Mar 5.

Department of Oncology and Hematology, Franz Tappeiner Hospital, Merano, Italy.

High-dose chemotherapy followed by autologous stem cell transplantation (HD-ASCT) as well as the introduction of novel agents (NA) significantly improved survival for patients with multiple myeloma (MM). A total of 150 unselected newly diagnosed MM patients treated at our institution from 1998 to 2017 were retrospectively analyzed. Median age at diagnosis was 69 years (range 33-93 years) with a median follow-up of 48. Read More

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http://dx.doi.org/10.1007/s00277-020-03969-9DOI Listing

Carfilzomib-induced hemolysis is noticeably common but rarely shows features of thrombotic microangiopathy: A retrospective study.

Eur J Haematol 2020 Jun 15;104(6):588-593. Epub 2020 Mar 15.

Division of Hematology, Department of Medicine, Faculty of Medicine and Health, Örebro University, Örebro, Sweden.

Objective: Hemolysis is a sporadically reported but potentially serious side effect of the proteasome inhibitor carfilzomib. We aimed to investigate the frequency of hemolysis in an unselected cohort.

Methods: We performed a retrospective, single-center study of the incidence of hemolysis in patients treated with carfilzomib, based mainly on consecutive haptoglobin levels. Read More

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http://dx.doi.org/10.1111/ejh.13401DOI Listing

Amyloid cardiomyopathy.

Authors:
Arnt V Kristen

Herz 2020 May;45(3):267-271

Kardiovaskuläres Zentrum Darmstadt, Ärztehaus 2 Mathildenhöhe, Dieburger Straße 31c, 64287, Darmstadt, Germany.

Cardiac amyloidosis is a heterogeneous group of diseases characterized by extracellular deposition of amyloid fibrils in many different organs finally resulting in organ failure. Cardiac involvement is common for immunoglobulin light chain amyloidosis (AL) or transthyretin amyloidosis (ATTR); the latter is caused by a transthyretin gene variant or wild-type protein. Precise diagnostic assessment including laboratory tests, electrocardiography, echocardiography, cardiac magnetic resonance imaging, biopsy, and/or bone scintigraphy is mandatory for definition of the amyloid type and finally for treatment initiation. Read More

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http://dx.doi.org/10.1007/s00059-020-04904-4DOI Listing

[The 474th case: anemia, ostealgia, proteinuria].

Zhonghua Nei Ke Za Zhi 2020 Feb;59(2):161-164

Department of Hematology, Multiple Myeloma Medical Center of Beijing, Beijing Chaoyang Hospital, Capital Medical University, Beijing 100043, China.

A 49-year-old woman was admitted to hospital with intermittent dizziness and fatigue for 7 years. The symptoms were aggravated and accompanied by bone pain for more than 4 months. She was referred to our hospital. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1426.2020.02.014DOI Listing
February 2020

Rare amyloidoma of the tongue base: A case report and review of the literature.

Mol Clin Oncol 2020 Mar 9;12(3):258-262. Epub 2020 Jan 9.

Department of Otorhinolaryngology, University of Medicine and Pharmacy of Craiova, 200349 Craiova, Romania.

Localized amyloidosis is a rare condition characterized by the deposition of misfolding protein in a tissue, without other systemic manifestations. Only a small number of cases of localized amyloidosis of the tongue have been reported to date, in contrast to systemic amyloidosis, in which localization on the tongue is common. This study presents a rare case of localized amyloidosis of the tongue (amyloidoma) and provides a summary of the known literature of localized amyloidosis. Read More

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http://dx.doi.org/10.3892/mco.2020.1972DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7016517PMC

Diagnostic imaging of cardiac amyloidosis.

Nat Rev Cardiol 2020 Feb 10. Epub 2020 Feb 10.

National Amyloidosis Centre, Division of Medicine, University College London, Royal Free Hospital, London, UK.

Systemic amyloidosis encompasses a debilitating, under-diagnosed but increasingly recognized group of disorders characterized by the extracellular deposition of misfolded proteins in one or more organs. Cardiac amyloid deposition leads to an infiltrative or restrictive cardiomyopathy and is the major contributor to poor prognosis in patients with systemic amyloidosis. In total, >30 proteins can form amyloid fibrils, but the two main types of amyloid that can infiltrate the heart are monoclonal immunoglobulin light-chain amyloid and transthyretin amyloid. Read More

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http://dx.doi.org/10.1038/s41569-020-0334-7DOI Listing
February 2020

Management of Cardiac Amyloidosis: Do's and Don'ts.

Can J Cardiol 2020 Mar 4;36(3):444-446. Epub 2019 Nov 4.

Stanford Amyloid Center, Division of Cardiovascular Medicine, Stanford University School of Medicine, Stanford, California, USA. Electronic address:

Cardiac amyloidosis is a potentially deadly disease characterized by progressive infiltration of amyloid fibrils, and it is increasingly recognized as an underdiagnosed but important cause of heart failure. Given its unique pathogenesis, there are key differences in the management of cardiac amyloidosis compared with other forms of heart failure. Moreover, the 2 common forms of cardiac amyloidosis, transthyretin and light-chain amyloidosis, are distinct entities with varying clinical manifestations and prognoses, leading to the need for tailored approaches to management. Read More

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http://dx.doi.org/10.1016/j.cjca.2019.10.032DOI Listing

Organ responses with daratumumab therapy in previously treated AL amyloidosis.

Blood Adv 2020 Feb;4(3):458-466

Deparment of Medicine, Stanford University School of Medicine, Stanford, CA and.

Immunoglobulin light chain amyloidosis (AL amyloidosis) involves deposition of abnormally folded light chains into a wide range of tissues causing organ dysfunction, including in the heart and kidney. Daratumumab, a CD38-targeted antibody, has recently demonstrated efficacy in producing hematologic responses in previously treated disease. However, data on survival outcomes and organ responses to daratumumab are lacking. Read More

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http://dx.doi.org/10.1182/bloodadvances.2019000776DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7013253PMC
February 2020

Melatonin attenuates vascular calcification by activating autophagy via an AMPK/mTOR/ULK1 signaling pathway.

Exp Cell Res 2020 Apr 1;389(1):111883. Epub 2020 Feb 1.

Department of Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing Institute of Heart Lung and Blood Vessel Disease, Beijing Key Laboratory of Precision Medicine of Coronary Atherosclerotic Disease, Clinical Center for Coronary Heart Disease, Capital Medical University, Beijing, China. Electronic address:

Melatonin has been demonstrated to protect against calcification in cyclosporine nephrotoxicity. Autophagy may affect vascular calcification by inhibiting apoptosis and the transdifferentiation process. This study sought to explore whether melatonin attenuates vascular calcification by regulating autophagy via the AMP-activated protein kinase/mammalian target of rapamycin/Unc-51-like kinase 1 (AMPK/mTOR/ULK1) signaling pathway. Read More

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http://dx.doi.org/10.1016/j.yexcr.2020.111883DOI Listing
April 2020
3.246 Impact Factor

[Effect of surgery of pulmonary cysts related to immunoglobulin light chain deposits].

Rev Mal Respir 2020 Feb 31;37(2):180-186. Epub 2020 Jan 31.

Service de pneumologie, centre hospitalier de Luxembourg, Strassen, Luxembourg.

Introduction: Light chain deposition disease is a rare anatomo-clinical disorder, which rarely leads to cystic lung destruction.

Case Report: We report the case of a 62years old female patient with a history of a monoclonal gammopathy of unknown significance who developed progressive dyspnea. Thoracic CT-scan demonstrated a diffuse pulmonary cystic disorder with predominance in the right lower lobe. Read More

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http://dx.doi.org/10.1016/j.rmr.2020.01.001DOI Listing
February 2020

Inhibition of Amyloid-Beta Production, Associated Neuroinflammation, and Histone Deacetylase 2-Mediated Epigenetic Modifications Prevent Neuropathology in Alzheimer's Disease Model.

Front Aging Neurosci 2019 15;11:342. Epub 2020 Jan 15.

Department of Immunology and Nano-Medicine, Institute of NeuroImmune Pharmacology, Herbert Wertheim College of Medicine, Florida International University, Miami, FL, United States.

Alzheimer's disease (AD) is a growing global threat to healthcare in the aging population. In the USA alone, it is estimated that one in nine persons over the age of 65 years is living with AD. The pathology is marked by the accumulation of amyloid-beta (Aβ) deposition in the brain, which is further enhanced by the neuroinflammatory process. Read More

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http://dx.doi.org/10.3389/fnagi.2019.00342DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6974446PMC
January 2020
2.843 Impact Factor

Serum amyloid P deposition is a sensitive and specific feature of membranous-like glomerulopathy with masked IgG kappa deposits.

Kidney Int 2020 Mar 9;97(3):602-608. Epub 2019 Nov 9.

University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA.

Membranous-like glomerulopathy with masked IgG kappa deposits (MGMID) is a recently described pattern of glomerulonephritis with a unique histopathology. The pattern is characterized by subepithelial and/or mesangial immune deposits that are "masked", to immunoglobulin staining by routine immunofluorescence but strongly stain for IgG and kappa light chain after protease digestion. Patients with this pattern of glomerulonephritis are most commonly young females presenting with proteinuria and a vague history of autoimmune disease such as low titer antinuclear antibodies. Read More

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http://dx.doi.org/10.1016/j.kint.2019.10.026DOI Listing
March 2020
8.563 Impact Factor

A Case of Primary Localized Small Bowel Amyloidosis Studied by 18F-Choline and Contrast-Enhanced 18F-FDG PET/CT.

Clin Nucl Med 2020 Mar;45(3):e154-e155

Istituto di Radiologia, Fondazione Policlinico Universitario A. Gemelli IRCCS, UOC di Radiologia, Dipartimento di Scienze Radiologiche, Radioterapiche ed Ematologiche Roma, Italia.

Amyloidosis is a rare hereditary or acquired protein deposition disorder with different etiologies, characterized by pathological protein deposition essentially in nearly any organs or tissues. There are 2 major forms: primary and secondary amyloidosis. Moreover, it is possible to have systemic or localized disease. Read More

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http://dx.doi.org/10.1097/RLU.0000000000002889DOI Listing

AMYLOID GOITER AS THE FIRST RECOGNIZABLE MANIFESTATION OF IMMUNOGLOBULIN LIGHT CHAIN AMYLOIDOSIS.

AACE Clin Case Rep 2019 Sep-Oct;5(5):e326-e329. Epub 2019 Jun 26.

Objective: Clinically apparent thyroid enlargement due to massive amounts of amyloid deposition, known as amyloid goiter, is rare. Endocrinologists should become familiar with this manifestation of systemic amyloidosis, which may be diagnosed by Congo red staining of the specimen obtained by fine-needle aspiration.

Methods: We describe a 70-year-old man who presented with a slowly enlarging goiter. Read More

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http://dx.doi.org/10.4158/ACCR-2019-0161DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6876951PMC

Human amyloidosis, still intractable but becoming curable: The essential role of pathological diagnosis in the selection of type-specific therapeutics.

Pathol Int 2020 Apr 21;70(4):191-198. Epub 2020 Jan 21.

Department of Amyloidosis Research, Nagasaki International University, Nagasaki, Japan.

The molecular pathogenesis of human amyloidosis has been elucidated greatly during the last 20 years. Based on the understanding of the molecular mechanisms of amyloid fibril formation and deposition, various kinds of new drugs and therapeutics have been emerging to improve the prognosis of amyloidosis and even cure this disease. In this review article, we first summarize the pathogenesis and state-of-the-art therapeutics of representative types of systemic human amyloidosis, that is, immunoglobulin light chain-related, transthyretin-related, amyloid A-associated and β -microglobulin-related amyloidosis. Read More

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http://dx.doi.org/10.1111/pin.12902DOI Listing

Renal amyloidosis complicated by light chain deposition nephropathy: a case report.

Int J Clin Exp Pathol 2019 1;12(6):2279-2283. Epub 2019 Jun 1.

Department of Nephrology, The First Affiliated Hospital of Xi'an Jiaotong University Xi'an, Shaanxi, China.

Light chain amyloidosis (AL) and light chain deposition disease (LCDD) are systemic diseases caused by an abnormal production of monoclonal immunoglobulin light chains and their deposition in systemic tissue. We herein present a rare case of renal amyloidosis complicated with light chain deposition nephropathy. The patient presented with nephrotic syndrome and an increased serum creatinine. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6949640PMC

Harnessing Cardiac Regeneration as a Potential Therapeutic Strategy for AL Cardiac Amyloidosis.

Curr Cardiol Rep 2020 Jan 13;22(1). Epub 2020 Jan 13.

Stanford Cardiovascular Institute, Stanford University School of Medicine, Stanford, CA, USA.

Purpose Of Review: Cardiac regeneration has received much attention as a possible means to treat various forms of cardiac injury. This review will explore the field of cardiac regeneration by highlighting the existing animal models, describing the involved molecular pathways, and discussing attempts to harness cardiac regeneration to treat cardiomyopathies.

Recent Findings: Light chain cardiac amyloidosis is a degenerative disease characterized by progressive heart failure due to amyloid fibril deposition and light chain-mediated cardiotoxicity. Read More

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http://dx.doi.org/10.1007/s11886-020-1252-3DOI Listing
January 2020

Simultaneous tubular and glomerular involvement with cryoglobulinemia vasculitis in multiple myeloma.

Saudi J Kidney Dis Transpl 2019 Nov-Dec;30(6):1470-1474

Department of Nephrology and Renal Transplantation, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India.

Renal manifestations in myeloma are varied. Tubulopathic light chains cause cast nephropathy or proximal tubulopathy, usually associated with tubulointerstitial nephritis. Glomerular involvement includes amyloidosis and monoclonal immunoglobulin deposition diseases. Read More

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http://dx.doi.org/10.4103/1319-2442.275496DOI Listing
January 2020

Recurrent pericardial effusion with pericardial amyloid deposition: a case report and literature review.

Cardiovasc Pathol 2020 May - Jun;46:107191. Epub 2019 Dec 6.

Department of Surgical Pathology, Tokyo Women's Medical University, Tokyo, Japan.

Pericardial amyloidosis is a rare cause of pericardial effusion. Here, we report a case of recurrent pericardial effusion because of pericardial amyloid deposition. The patient was a man in his 40s admitted for pulmonary embolism. Read More

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http://dx.doi.org/10.1016/j.carpath.2019.107191DOI Listing
December 2019

A Case of Severe Cholestasis due to Hepatic AL Amyloidosis.

GE Port J Gastroenterol 2019 Oct 7;26(6):425-429. Epub 2019 Feb 7.

Department of Internal Medicine, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

Introduction: Immunoglobulin light chain-associated amyloidosis results from extracellular tissue deposition of fibril-forming monoclonal immunoglobulin light chains, secreted by a clone of plasma cells. Although the liver is often involved histologically, most cases are clinically asymptomatic, and severe intrahepatic cholestasis as the primary manifestation of the disease is rare.

Clinical Case: We report an unusual case of primary amyloidosis in a 71-year-old man, presenting with rapidly progressive cholestasis, associated with hepatomegaly. Read More

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http://dx.doi.org/10.1159/000496185DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6876644PMC
October 2019

Epidemiological and clinical characteristics and outcome of monoclonal gammopathy of renal significance-related lesions in Latin America.

Nephrology (Carlton) 2020 Jun 19;25(6):442-449. Epub 2019 Dec 19.

Department of Pathology, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago de, Chile.

Background: Monoclonal gammopathy of renal significance (MGRS)-related lesions are infrequent entities. There are no publications on these disorders in Latin America (LA). The aim of this study was to describe epidemiological and clinical characteristics of these patients in LA. Read More

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http://dx.doi.org/10.1111/nep.13685DOI Listing
June 2020
1.864 Impact Factor

Light chain amyloidosis induced inflammatory changes in cardiomyocytes and adipose-derived mesenchymal stromal cells.

Leukemia 2020 May 3;34(5):1383-1393. Epub 2019 Dec 3.

Department of Immunology, Mayo Clinic, Rochester, MN, USA.

Light chain (AL) amyloidosis is a progressive, degenerative disease characterized by the misfolding and amyloid deposition of immunoglobulin light chain (LC). The amyloid deposits lead to organ failure and death. Our laboratory is specifically interested in cardiac involvement of AL amyloidosis. Read More

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http://dx.doi.org/10.1038/s41375-019-0640-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7196017PMC