854 results match your criteria Light-Chain Deposition Disease


Light-Chain Deposition Disease Presenting with Spontaneous Splenic Rupture.

Eur J Case Rep Intern Med 2018 27;5(12):0001010. Epub 2018 Dec 27.

Serviço de Medicina 1, ULSAM, Viana do Castelo, Portugal.

Light-chain deposition disease is a rare paraproteinaemia characterized by deposition of monoclonal light-chain immunoglobulins with a non-amyloid structure, most frequently affecting the kidney. The authors present the case of a 58-year-old man admitted due to haemorrhagic shock caused by spontaneous splenic rupture. Investigation showed coagulopathy, homogeneous hepatomegaly with elevated cholestasis enzymes but normal bilirubinaemia, and a nephrotic syndrome with monoclonal kappa light chains. Read More

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http://dx.doi.org/10.12890/2018_0001010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346975PMC
December 2018
1 Read

Perianal purpuric plaques revealing an amyloid light-chain amyloidosis: case report and review of the literature.

Dermatol Online J 2019 Jan 15;25(1). Epub 2019 Jan 15.

Department of Dermatology and Venereology, Centro Hospitalar São João, Porto.

Systemic immunoglobulin light chain amyloidosis is the most common and severe type of amyloidosis. There is an abnormal fibrillary protein deposition in tissues that leads to progressive and irreversible organ dysfunction. The most commonly affected organs are kidney and heart. Read More

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January 2019
2 Reads

[A case of bronchiectasis due to light chain deposition disease].

Rev Mal Respir 2019 Jan 24. Epub 2019 Jan 24.

Département de pneumologie, hôpitaux de Brabois, CHU de Nancy, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France.

Introduction: The natural history of orphan lung diseases is often unclear. We report the long-term follow-up of a case of bronchiectasis due to pulmonary non amyloid light chain deposition disease (LCDD).

Case Report: A 50-year-old woman who was a smoker, was diagnosed with diffuse thin walled bronchiectasis of uncertain origin after presenting with a respiratory tract infection. Read More

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http://dx.doi.org/10.1016/j.rmr.2018.11.008DOI Listing
January 2019
1 Read

Serum neurofilament dynamics predicts neurodegeneration and clinical progression in presymptomatic Alzheimer's disease.

Nat Med 2019 Feb 21;25(2):277-283. Epub 2019 Jan 21.

German Center for Neurodegenerative Diseases (DZNE), Tübingen, Germany.

Neurofilament light chain (NfL) is a promising fluid biomarker of disease progression for various cerebral proteopathies. Here we leverage the unique characteristics of the Dominantly Inherited Alzheimer Network and ultrasensitive immunoassay technology to demonstrate that NfL levels in the cerebrospinal fluid (n = 187) and serum (n = 405) are correlated with one another and are elevated at the presymptomatic stages of familial Alzheimer's disease. Longitudinal, within-person analysis of serum NfL dynamics (n = 196) confirmed this elevation and further revealed that the rate of change of serum NfL could discriminate mutation carriers from non-mutation carriers almost a decade earlier than cross-sectional absolute NfL levels (that is, 16. Read More

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http://dx.doi.org/10.1038/s41591-018-0304-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367005PMC
February 2019
9 Reads

Symptomatic involvement of the stomach and duodenum as initial presentation of AL amyloidosis.

BMJ Case Rep 2019 Jan 17;12(1). Epub 2019 Jan 17.

Cleveland Clinic Florida, Weston, Florida, USA.

Primary systemic or amyloid light chain (AL) amyloidosis is a multisystem disorder with myriad presentations. Although it can involve the upper gastrointestinal tract in 8% of cases, symptomatic involvement is exceedingly rare. We chronicle here a case of postprandial epigastric pain associated with AL amyloidosis involving the stomach and duodenum. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22755
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http://dx.doi.org/10.1136/bcr-2018-227550DOI Listing
January 2019
8 Reads

Recent Advances in the Diagnosis, Risk Stratification, and Management of Systemic Light-Chain Amyloidosis.

Acta Haematol 2019 Jan 16;141(2):93-106. Epub 2019 Jan 16.

Division of Hematology, Mayo Clinic, Rochester, Minnesota,

The term amyloidosis refers to a group of disorders in which protein fibrils accumulate in certain organs, disrupt their tissue architecture, and impair the function of the effected organ. The clinical manifestations and prognosis vary widely depending on the specific type of the affected protein. Immunoglobulin light-chain (AL) amyloidosis is the most common form of systemic amyloidosis, characterized by deposition of a misfolded monoclonal light-chain that is secreted from a plasma cell clone. Read More

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http://dx.doi.org/10.1159/000495455DOI Listing
January 2019
1 Read

[Diagnosis and treatment of kidney involvement in plasma cell diseases : Renal involvement in multiple myeloma and monoclonal gammopathies].

Internist (Berl) 2019 Jan;60(1):10-22

Klinik für Nieren- und Hochdruckerkrankungen, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland.

Background: Kidney involvement is a common complication in patients with plasma cell diseases.

Objective: This article outlines the spectrum of renal involvement in plasma cell dyscrasia and describes diagnostic and therapeutic measures to guide clinical management.

Material And Methods: Evaluation and discussion of the current literature as well as existing guidelines and recommendations of professional societies. Read More

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http://dx.doi.org/10.1007/s00108-018-0538-7DOI Listing
January 2019
2 Reads

Localized Lichen Myxedematosus With Plasma Cell Light Chain Restriction. Is It the Exception or the Rule?

Am J Dermatopathol 2018 Dec 20. Epub 2018 Dec 20.

Global Pathology Laboratory, Aurora Diagnostics, Miami Lakes, FL.

Lichen myxedematosus is a chronic cutaneous mucinosis that can present on a spectrum from localized cutaneous lesions to systemic disease of scleromyxedema. The clinical presentation of localized cutaneous lichen myxedematosus is waxy lichenoid papules, nodules, and/or plaques that have histopathologic findings of mucin deposition and a variable degree of fibroblast proliferation. There is an absence of serum paraproteins, and there are no other systemic causes of cutaneous mucinosis such as thyroid disease. Read More

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http://dx.doi.org/10.1097/DAD.0000000000001335DOI Listing
December 2018
3 Reads

Lingual liability: macroglossia and dyspnoea as the harbinger of systemic AL (light-chain) cardiac amyloidosis.

BMJ Case Rep 2018 Dec 22;11(1). Epub 2018 Dec 22.

Division of Cardiology, Tripler Army Medical Center, Tripler, Hawaii, USA.

A 58-year-old man presented with a chief complaint of tongue indentations and discomfort. Otolaryngology treated him for oral thrush with counselling to avoid tongue biting. In addition, the patient reported dyspnoea described as a decrease in tolerance of his physical activities. Read More

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http://dx.doi.org/10.1136/bcr-2018-225923DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6307669PMC
December 2018
3 Reads

Randall-type monoclonal immunoglobulin deposition disease: novel insights from a nationwide cohort study.

Blood 2019 Feb 21;133(6):576-587. Epub 2018 Dec 21.

Department of Nephrology, CHU Poitiers, Poitiers, France.

Monoclonal immunoglobulin deposition disease (MIDD) is a rare complication of B-cell clonal disorders, defined by Congo red negative-deposits of monoclonal light chain (LCDD), heavy chain (HCDD), or both (LHCDD). MIDD is a systemic disorder with prominent renal involvement, but little attention has been paid to the description of extrarenal manifestations. Moreover, mechanisms of pathogenic immunoglobulin deposition and factors associated with renal and patient survival are ill defined. Read More

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http://dx.doi.org/10.1182/blood-2018-09-872028DOI Listing
February 2019
1 Read

Clinical and endoscopic manifestations of gastrointestinal amyloidosis: a case series.

Clujul Med 2018 Oct 30;91(4):469-473. Epub 2018 Oct 30.

Gastroenterology and Hepatology Department, Mayo Clinic Florida, United States.

Gastrointestinal (GI) amyloidosis is rare and has varying clinical and endoscopic presentations. In this case series, we present three patients with primary systemic amyloid-light chain (AL) amyloidosis with GI involvement and complications of GI bleeding. We also provide a brief review of the literature, including clinical presentation, endoscopic findings, pathology, and management of GI amyloidosis. Read More

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http://dx.doi.org/10.15386/cjmed-951DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6296722PMC
October 2018
1 Read

Complete remission of both immunoglobulin light chain amyloidosis and psoriasis after autologous hematopoietic stem cell transplantation: A case report.

Medicine (Baltimore) 2018 Dec;97(50):e13589

National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, Nanjing, Jiangsu, China.

Rationale: Immunoglobulin light chain amyloidosis (AL amyloidosis) is characterized by the deposition of abnormal amyloid protein produced by a pathological plasma cell clone in various organs and soft tissues. Hematopoietic stem cell transplantation (HSCT) is an effective way to treat AL amyloidosis. Psoriasis is a common autoimmune disease (AID) and HSCT is a potential treatment for severe AIDs. Read More

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http://dx.doi.org/10.1097/MD.0000000000013589DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6320088PMC
December 2018
1 Read

[Corneal deposits in monoclonal gammopathy of undetermined significance. Review of the literature and case report].

Orv Hetil 2018 Sep;159(39):1575-1583

Szemészeti Klinika, Semmelweis Egyetem, Általános Orvostudományi Kar Budapest.

To summarize ophthalmological signs of monoclonal gammopathy of undetermined significance (MGUS) and to present a case report. Summary of the literature data and presentation of the history of a 46-year-old female patient. In MGUS, pathological, but non-malignant plasma cells produce abnormal monoclonal immunoglobulin. Read More

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http://dx.doi.org/10.1556/650.2018.31196DOI Listing
September 2018
2 Reads

Treatment of multiple myeloma with renal involvement: the nephrologist's view.

Clin Kidney J 2018 Dec 1;11(6):777-785. Epub 2018 Aug 1.

Nephrology Department, Hospital Universitari de Bellvitge, Idibell, Barcelona, Spain.

Renal injury is a common complication in multiple myeloma (MM). In fact, as many as 10% of patients with MM develop dialysis-dependent acute kidney injury related to increased free light chain (FLC) production by a plasma cell clone. Myeloma cast nephropathy (MCN) is the most prevalent pathologic diagnosis associated with renal injury, followed by light chain deposition disease and light chain amyloidosis. Read More

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http://dx.doi.org/10.1093/ckj/sfy065DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6275441PMC
December 2018
1 Read

AL amyloidosis with non-amyloid forming monoclonal immunoglobulin deposition; a case mimicking AHL amyloidosis.

BMC Nephrol 2018 Nov 22;19(1):337. Epub 2018 Nov 22.

Kidney and Vascular Pathology, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan.

Background: Immunoglobulin heavy-and-light-chain amyloidosis (AHL amyloidosis) is a newly established disease entity where both the immunoglobulin heavy-chain and light-chain compose amyloid fibrils. The immunoglobulins responsible for the amyloid fibrils are generally identified by immunostaining and/or laser microdissection-liquid chromatography-tandem mass spectrometry (LMD-LC-MS/MS). However, both techniques do not biochemically differentiate immunoglobulins that formed amyloid fibrils from non-responsible immunoglobulins. Read More

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http://dx.doi.org/10.1186/s12882-018-1050-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6251104PMC
November 2018
11 Reads

Primary idiopathic CNS non-amyloidogenic light chain deposition disease complicated by treatment-resistant focal seizure disorder.

J Clin Neurosci 2019 Jan 10;59:313-315. Epub 2018 Nov 10.

Department of Neurology, Auckland City Hospital, Auckland, New Zealand.

Light chain deposition disease (LCDD) is a systemic disorder characterised by the pathologic deposition of immunoglobulin light chains, which is histologically distinguished from amyloidosis by failure to stain with Congo red. Central nervous system (CNS)-restricted LCDD is among the rarest manifestations. We describe a unique case complicated by focal onset epilepsy with impaired awareness for which control with anticonvulsant therapy proved difficult. Read More

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http://dx.doi.org/10.1016/j.jocn.2018.10.123DOI Listing
January 2019
1 Read

Monoclonal light chain crystalline podocytopathy and tubulopathy associated with monoclonal gammopathy of renal significance: a case report and literature review.

BMC Nephrol 2018 Nov 12;19(1):322. Epub 2018 Nov 12.

Renal Division, Department of Medicine, Peking University First Hospital, Beijing, 100034, People's Republic of China.

Background: Monoclonal gammopathy of renal significance (MGRS) is a recently defined group of renal diseases caused by monoclonal immunoglobulin secreted by nonmalignant proliferative B cell or plasma cell. Monoclonal immunoglobulin can form different types of structures deposited in renal tissue, including fibrils, granules, microtubules, crystals and casts, and has mostly been reported in multiple myeloma patients. Here we report a rare case with κ light chain crystals in both podocytes and tubular epithelial cells associated with MGRS, which adds more information to the spectrum of MGRS-related renal diseases. Read More

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https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882
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http://dx.doi.org/10.1186/s12882-018-1108-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6233383PMC
November 2018
7 Reads
1.520 Impact Factor

Isolation and purification of recombinant immunoglobulin light chain variable domains from the periplasmic space of Escherichia coli.

PLoS One 2018 22;13(10):e0206167. Epub 2018 Oct 22.

Institute of Integrative Biology, University of Liverpool, Liverpool, United Kingdom.

Immunoglobulin light chain amyloidosis is the most common form of systemic amyloidosis. However, very little is known about the underlying mechanisms that initiate and modulate the associated protein aggregation and deposition. Model systems have been established to investigate these disease-associated processes. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0206167PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6197867PMC
October 2018
9 Reads

Long-Term Follow-Up of 4 Patients with Conjunctival Amyloidosis.

Ocul Oncol Pathol 2018 Sep 13;4(5):313-317. Epub 2018 Feb 13.

Department of Ophthalmology, Memorial Sloan Kettering Cancer Center, New York, New York, USA.

Conjunctival amyloidosis is a rare cause of ocular inflammation, mass, and hemorrhage that can be difficult to diagnose and treat. In this case series, we describe 4 patients with a histopathological diagnosis of conjunctival amyloidosis treated at a single institution. All patients underwent surgical excision and biopsy. Read More

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https://www.karger.com/Article/FullText/485918
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http://dx.doi.org/10.1159/000485918DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6167687PMC
September 2018
14 Reads

[Amyloid Registry Kiel : An interim balance].

Authors:
C Röcken

Pathologe 2018 Dec;39(Suppl 2):301-305

Institut für Pathologie, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Christian-Albrechts-Universität Kiel, Arnold-Heller-Straße 3/14, 24105, Kiel, Deutschland.

Amyloid is a pathologic fibrillar aggregation of polypeptides in a cross-β-sheet conformation. Amyloidoses are caused by the deposition of amyloid and may occur as cerebal and extracerebral diseases. A total of 35 different amyloid proteins have been identified. Read More

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http://dx.doi.org/10.1007/s00292-018-0515-xDOI Listing
December 2018
4 Reads

AL amyloidosis: advances in diagnostics and treatment.

Authors:
Romana Ryšavá

Nephrol Dial Transplant 2018 Oct 8. Epub 2018 Oct 8.

Department of Nephrology, First Faculty of Medicine, Charles University, Prague, Czech Republic.

AL amyloidosis (light chain; previously also called primary amyloidosis) is a systemic disease characterized by an amyloid deposition process affecting many organs, and which still has unsatisfactory survival of patients. The monoclonal light chains kappa (κ) or lambda (λ) or their fragments form the fibrils that deposit and accumulate in different tissues. Renal involvement is very frequent in AL amyloidosis and can lead to the development of nephrotic syndrome followed by renal failure in some cases. Read More

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http://dx.doi.org/10.1093/ndt/gfy291DOI Listing
October 2018
4 Reads

Chronic intestinal pseudo-obstruction due to al amyloidosis: a case report and literature review.

Clin J Gastroenterol 2018 Sep 19. Epub 2018 Sep 19.

Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine, Minami 1-Jo Nishi 16-Chome, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan.

A 59-year-old woman presented to our hospital with a 6-month history of nausea, weight loss, and abdominal distension. Physical examination revealed abdominal distension without tenderness, and edema, numbness, and multiple peripheral neuropathy in the limbs. Blood test results showed anemia, hypoproteinemia, and hypoalbuminemia. Read More

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http://dx.doi.org/10.1007/s12328-018-0909-6DOI Listing
September 2018
5 Reads

Acquired Fanconi syndrome secondary to light chain deposition disease associated with monoclonal gammopathy of renal significance: A case report.

Medicine (Baltimore) 2018 Sep;97(36):e12027

Department of Nephrology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang.

Rationale: Renal Fanconi syndrome (FS) is a rare complication of monoclonal gammopathy. It is characterized by the impairment of renal proximal tubular function leading to normoglycemic glycosuria, aminoaciduria, hypophosphatemia, hypouricemia and proximal renal tubular acidosis. Renal impairment in monoclonal gammopathy, without fulfilling the criteria of multiple myeloma, is categorized as monoclonal gammopathy of renal significance (MGRS). Read More

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http://Insights.ovid.com/crossref?an=00005792-201809070-0002
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http://dx.doi.org/10.1097/MD.0000000000012027DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6133467PMC
September 2018
7 Reads

Pemphigus vulgaris as the first manifestation of multiple myeloma: a case report.

J Med Case Rep 2018 Sep 7;12(1):255. Epub 2018 Sep 7.

Department of Dermatology, University Hospital Joseph Raseta Befelatanana, Antananarivo, Madagascar.

Background: The association between pemphigus and malignancy has been well documented for decades but an association between pemphigus vulgaris and multiple myeloma is unusual. We report a case of pemphigus vulgaris revealing multiple myeloma.

Case Presentation: A 55-year-old Malagasy man, with no significant past medical history, presented with bullous and erosive skin lesions involving his trunk and scalp for the last 2 months. Read More

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http://dx.doi.org/10.1186/s13256-018-1791-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6127896PMC
September 2018
9 Reads

Unmasking and successful management of light chain deposition disease of kidney in pregnancy: a complex case, mirroring the complex needs of pregnancy with kidney disease in India.

J Nephrol 2018 Oct 5;31(5):785-791. Epub 2018 Sep 5.

Department of Nephrology, Christian Medical College and Hospital, Vellore, Tamil Nadu, 632004, India.

Pregnancy offers a precious window of opportunity to diagnose previously undetected or new onset kidney diseases in emerging countries like India, where access to medical, educational and health care facilities are not equitably distributed across varied sections of society. We report a case of a 33 year-old primi gravida who had a successful pregnancy following what was initially considered to represent preeclampsia at 38 weeks of gestation, in whom a subsequent kidney biopsy for persistence of pregnancy-related acute kidney injury (Pr-AKI) revealed light chain deposition disease (LCDD). The etiological evaluation of LCDD led to the detection of an underlying plasma cell dyscrasia which was treated effectively with chemotherapy and autologous stem cell transplant. Read More

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http://link.springer.com/10.1007/s40620-018-0532-0
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http://dx.doi.org/10.1007/s40620-018-0532-0DOI Listing
October 2018
11 Reads
2.000 Impact Factor

A Case of Switch from C3 Glomerulonephritis to Proliferative Glomerulonephritis with Monoclonal IgG Deposits.

Ann Clin Lab Sci 2018 Jul;48(4):528-533

National Clinical Research Center of Kidney Disease, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China

Monoclonal immunoglobulins have been implicated in the development of C3 glomerulonephritis (C3GN) and Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID). We report a 58-year-old female who showed a switch from C3GN to PGNMID. She presented with mild proteinuria and normal renal function for the first time. Read More

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July 2018
4 Reads

Lenalidomide as a Beneficial Treatment Option for Renal Impairment Caused by Light Chain Deposition Disease.

Intern Med 2018 Dec 10;57(24):3651-3657. Epub 2018 Aug 10.

Department of Hematology, Fukushima Medical University, Japan.

Light chain deposition disease (LCDD) is a rare systemic disorder caused by the deposition of light chain immunoglobulins, which often results in renal impairment associated with either nephrotic syndrome or asymptomatic proteinuria. B-cell neoplasms, such as multiple myeloma and lymphoproliferative disorders, are well-known underlying diseases in LCDD. Some chemotherapy regimens have been reported, but both evidence-based treatment and management for LCDD have yet to be established. Read More

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http://dx.doi.org/10.2169/internalmedicine.1018-18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6355407PMC
December 2018
8 Reads

The effects of subacute inhaled multi-walled carbon nanotube exposure on signaling pathways associated with cholesterol transport and inflammatory markers in the vasculature of wild-type mice.

Toxicol Lett 2018 Oct 3;296:48-62. Epub 2018 Aug 3.

Advanced Environmental Research Institute, Department of Biological Sciences, University of North Texas, Denton, TX, 76201, USA. Electronic address:

Exposure to multi-walled carbon nanotubes (MWCNTs) has been associated with detrimental cardiovascular outcomes; however, underlying mechanisms have not yet been fully elucidated. Thus, we investigated alterations in proatherogenic and proinflammatory signaling pathways in C57Bl6/ mice exposed to MWCNTs (1 mg/m) or filtered air (FA-Controls), via inhalation, for 6 h/day, 14d. Expression of mediators of cholesterol transport, namely the lectin-like oxidized low-density lipoprotein receptor (LOX)-1 and ATP-binding cassette transporter (ABCA)-1, inflammatory markers tumor necrosis factor (TNF)-α and interleukin (IL)-1β/IL-6, nuclear-factor kappa-light-chain-enhancer of activated B cells (NF-κB), intracellular/vascular adhesion molecule(s) (VCAM-1, ICAM-1), and miRNAs (miR-221/-21/-1), associated with cardiovascular disease (CVD), were analyzed in cardiac tissue and coronary vasculature. Read More

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http://dx.doi.org/10.1016/j.toxlet.2018.08.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6126380PMC
October 2018
27 Reads

Histopathologic findings in lung biopsies from patients with primary biliary cholangitis.

Hum Pathol 2018 Dec 29;82:177-186. Epub 2018 Jul 29.

Division of Anatomic Pathology, Mayo Clinic, Rochester, 55905 MN. Electronic address:

Primary biliary cholangitis (PBC) is a progressive autoimmune disease of the liver causing destruction of intrahepatic bile ducts, associated with lymphocytic and granulomatous inflammation. PBC has been associated with many extrahepatic manifestations including interstitial lung disease. However, comprehensive pulmonary histopathology in PBC has not been well documented. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00468177183028
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http://dx.doi.org/10.1016/j.humpath.2018.07.021DOI Listing
December 2018
6 Reads

Retinoic Acid Receptor-β Is Downregulated in Hepatocellular Carcinoma and Cirrhosis and Its Expression Inhibits Myosin-Driven Activation and Durotaxis in Hepatic Stellate Cells.

Hepatology 2019 Feb 17;69(2):785-802. Epub 2018 Dec 17.

Cellular and Molecular Biomechanics Laboratory, Department of Bioengineering, Imperial College London, London, United Kingdom.

Hepatic stellate cells (HSCs) are essential perisinusoidal cells in both healthy and diseased liver. HSCs modulate extracellular matrix (ECM) homeostasis when quiescent, but in liver fibrosis, HSCs become activated and promote excess deposition of ECM molecules and tissue stiffening via force generation and mechanosensing. In hepatocellular carcinoma (HCC), activated HSCs infiltrate the stroma and migrate to the tumor core to facilitate paracrine signaling with cancer cells. Read More

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http://dx.doi.org/10.1002/hep.30193DOI Listing
February 2019
9 Reads

Biventricular assessment of light-chain amyloidosis using 3D speckle tracking echocardiography: Differentiation from other forms of myocardial hypertrophy.

Int J Cardiol 2018 Nov 23;271:371-377. Epub 2018 Jul 23.

Department of Cardiology, Sapienza University, Rome, Italy; Department of Hematology, Sapienza University, Rome, Italy; Department of Medicine, Sapienza University, Rome, Italy.

Background: Given that in patients with cardiac amyloidosis (CA), deposition of amyloid protein is not restricted to the left ventricular (LV) myocardium, it can be hypothesized that the diagnostic value of deformation mechanics would be enhanced by considering right ventricular (RV) strain measures. The aim of the present study was to examine the potential utility of left ventricular (LV) and right ventricular (RV) deformation and rotational parameters derived from three-dimensional speckle-tracking echocardiograph (3DSTE) to diagnose cardiac amyloidosis and differentiate this disease from other forms of myocardial hypertrophy.

Methods: Twenty-three patients with biopsy-proven light-chain (AL) amyloidosis, 23 patients with systemic arterial hypertension (HTN), 23 patients with hypertrophic cardiomyopathy (HCM), 23 athletes and 23 normal controls were prospectively studied by conventional echocardiography and 3DSTE. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01675273173638
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http://dx.doi.org/10.1016/j.ijcard.2018.03.088DOI Listing
November 2018
16 Reads

Pressure Overload in Mice With Haploinsufficiency of Striated Preferentially Expressed Gene Leads to Decompensated Heart Failure.

Front Physiol 2018 10;9:863. Epub 2018 Jul 10.

Division of Pulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, United States.

Striated preferentially expressed gene (Speg) is a member of the myosin light chain kinase family of proteins. Constitutive Speg deficient (Speg) mice develop a dilated cardiomyopathy, and the majority of these mice die or shortly after birth. In the present study we assessed the importance of Speg in adult mice. Read More

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http://dx.doi.org/10.3389/fphys.2018.00863DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6048438PMC
July 2018
4 Reads

Immunoglobulin light chain amyloidosis: 2018 Update on diagnosis, prognosis, and treatment.

Authors:
Morie A Gertz

Am J Hematol 2018 Sep;93(9):1169-1180

Division of Hematology, Mayo Clinic, Rochester, Minnesota.

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 restrictive cardiomyopathy, nephrotic syndrome, hepatic dysfunction, peripheral/autonomic neuropathy, and "atypical multiple myeloma."

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.25149DOI Listing
September 2018
36 Reads

European myeloma network recommendations on diagnosis and management of patients with rare plasma cell dyscrasias.

Leukemia 2018 Sep 23;32(9):1883-1898. Epub 2018 Jul 23.

Department of Clinical Therapeutics, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece.

The introduction of novel agents in the management of multiple myeloma and related plasma cell dyscrasias has changed our treatment approaches and subsequently the outcome of patients. Due to current advances, the European Myeloma Network updated the diagnostic and therapeutic recommendations for patients with Waldenström's macroglobulinemia (WM), AL-amyloidosis, monoclonal immunoglobulin deposition disease (MIDD), POEMS syndrome, and primary plasma cell leukemia. For patients with WM, the combination of rituximab with chemotherapy remains the treatment cornerstone, while the Bruton-tyrosine kinase inhibitor ibrutinib has been introduced and approved for relapsed/refractory disease. Read More

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http://www.nature.com/articles/s41375-018-0209-7
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http://dx.doi.org/10.1038/s41375-018-0209-7DOI Listing
September 2018
12 Reads

Gastroparesis in a Patient with Gastric AL Amyloidosis.

Case Rep Gastroenterol 2018 May-Aug;12(2):317-321. Epub 2018 Jun 22.

Department of Gastroenterology, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio, USA.

Systemic amyloidosis is a group of complex disorders characterized by the extracellular deposition of nonbranching fibrils in various tissues, ultimately leading to a variety of clinical presentations including isolated or multiorgan dysfunction. Amyloid involvement of the gastrointestinal tract is common depending on the subtype of this disease; light chain primary amyloidosis (AL) and secondary amyloidosis (AA) affect the gastrointestinal tract in unique ways due to differing pathophysiology. A case is reported of gastroparesis associated with AL amyloidosis diagnosed by esophagogastroduodenoscopy and study of gastric emptying, then subsequently confirmed by Congo red staining performed on endoscopic biopsies. Read More

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http://dx.doi.org/10.1159/000489297DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6047544PMC
June 2018
3 Reads

Multisystem amyloidosis as the unifying diagnosis for constipation, collapse and cardiomyopathy.

BMJ Case Rep 2018 Jul 10;2018. Epub 2018 Jul 10.

Gastroenterology, University Hospitals Coventry and Warwickshire NHS Trust, Coventry, UK.

Amyloidosis a rare disorder characterised by the deposition of amyloid protein aggregates in different organ systems throughout the body with resulting functional impairment of affected organs. It can present with localised or multisystemic deposits. Diagnosis is often delayed due to the non-specific nature of the symptoms. Read More

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http://dx.doi.org/10.1136/bcr-2018-225301DOI Listing
July 2018
10 Reads

Behçet disease associated with gastrointestinal amyloidosis manifested as hematochezia: A case report.

Medicine (Baltimore) 2018 Jun;97(26):e11153

Department of Rheumatology.

Rationale: Behçet disease (BD) is an inflammatory disorder characterized by recurrent oral aphthous ulcers, genital ulcers, ocular lesions, and skin lesions. Complication of amyloidosis in patients with BD is rare. Here, we report a case of BD with immunoglobulin light chain (AL)-amyloidosis manifested as hematochezia. Read More

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http://dx.doi.org/10.1097/MD.0000000000011153DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6039685PMC
June 2018
4 Reads

Phenotypic characterization of murine models of cerebral cavernous malformations.

Lab Invest 2018 Jun 26. Epub 2018 Jun 26.

Neurovascular Surgery Program, Section of Neurosurgery, The University of Chicago Medicine and Biological Sciences, Chicago, IL, USA.

Cerebral cavernous malformations (CCMs) are clusters of dilated capillaries that affect around 0.5% of the population. CCMs exist in two forms, sporadic and familial. Read More

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http://dx.doi.org/10.1038/s41374-018-0030-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6309944PMC
June 2018
18 Reads

Collapsing Glomerulopathy in Lambda Light Chain Amyloidosis: A Report of 2 Cases.

Am J Kidney Dis 2018 Oct 19;72(4):612-616. Epub 2018 Jun 19.

Sharp Memorial Hospital, San Diego, CA.

Amyloid nephropathy is an uncommon disease that frequently presents with reduced kidney function and proteinuria and, in developed nations, is most often associated with underlying paraproteinemia. The histologic appearance of glomerular amyloid deposition includes mesangial and capillary wall infiltration by an amorphous eosinophilic material, and features of endo- or extracapillary proliferation are not typically seen. Rare cases of crescentic injury have been reported in a subset of patients with amyloid nephropathy, particularly those with amyloid derived from serum amyloid A protein. Read More

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http://dx.doi.org/10.1053/j.ajkd.2018.04.009DOI Listing
October 2018
7 Reads

External beam radiation for localized periocular amyloidosis: a case series.

Orbit 2018 Jun 11:1-7. Epub 2018 Jun 11.

d Department of Ophthalmology and Visual Sciences , Vanderbilt University Medical Center , Nashville , TN , USA.

Purpose: To evaluate the effectiveness of low dose external beam radiation therapy to halt progression of localized periocular light chain (AL) amyloidosis, a clonal plasma cell disorder.

Methods: This is a retrospective review of patients referred to a tertiary care center for external beam radiation treatment of biopsy proven localized periocular light chain amyloidosis. The primary outcome measure was clinical disease stability at one year following radiation therapy as evidenced by slit lamp exam and external photography. Read More

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http://dx.doi.org/10.1080/01676830.2018.1483407DOI Listing
June 2018
2 Reads

Amyloid Proximal Tubulopathy and Amyloid Casts: An Unusual Finding in Multiple Myeloma.

Indian J Nephrol 2018 Mar-Apr;28(2):160-163

Department of Nephrology, Government Stanley Medical College, Chennai, Tamil Nadu, India.

Patients with multiple myeloma (MM) often develop renal manifestations. The majority of cases present as cast nephropathy, amyloid light-chain (AL) amyloidosis, and monoclonal immunoglobulin deposition disease. AL amyloidosis usually involves the glomeruli, blood vessels, and interstitium. Read More

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http://dx.doi.org/10.4103/ijn.IJN_297_16DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5952456PMC
June 2018
2 Reads

Sjögren Syndrome Complicated with Cystic Lung Disease and Pulmonary Amyloidosis.

Case Rep Rheumatol 2018 31;2018:7475242. Epub 2018 Mar 31.

Division of Hematology, Respiratory Medicine and Oncology, Department of Internal Medicine, Faculty of Medicine, Saga University, 5-1-1 Nabeshima, Saga 849-8501, Japan.

A 72-year-old Japanese woman was noted to have multiple cystic lung shadows and infiltrates on chest radiography and computed tomography (CT). She complained of dryness of the mouth and eyes, but she did not have respiratory symptoms, such as cough, sputum production, and dyspnea. Her laboratory findings showed high titers of anti-SSA/Ro and anti-SSB/La antibodies. Read More

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http://dx.doi.org/10.1155/2018/7475242DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5901953PMC
March 2018
6 Reads

Management of the elderly patient with AL amyloidosis.

Eur J Intern Med 2018 12 23;58:48-56. Epub 2018 May 23.

Amyloidosis Research and Treatment Center, Foundation IRCCS Policlinico San Matteo, Department of Molecular Medicine, University of Pavia, Italy. Electronic address:

Systemic immunoglobulin light chain (AL) amyloidosis is an aging-associated protein misfolding and deposition disease. This condition is caused by a small and otherwise indolent plasma cell (or B cell) clone secreting an unstable circulating light chain, which misfolds and deposits as amyloid fibrils possibly leading to progressive dysfunction of affected organs. AL amyloidosis can occur in the typical setting of other, rarer forms of systemic amyloidosis and can mimic other more prevalent conditions of the elderly. Read More

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http://dx.doi.org/10.1016/j.ejim.2018.05.004DOI Listing
December 2018
7 Reads

Atorvastatin Inhibits Inflammatory Response, Attenuates Lipid Deposition, and Improves the Stability of Vulnerable Atherosclerotic Plaques by Modulating Autophagy.

Front Pharmacol 2018 3;9:438. Epub 2018 May 3.

Department of Cardiology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.

Atherosclerosis is a chronic disease comprising intima malfunction and arterial inflammation. Recent studies have demonstrated that autophagy could inhibit inflammatory response in atherosclerosis and exert subsequent atheroprotective effects. Our previous study also demonstrated the role of autophagy in the inhibition of inflammation by atorvastatin . Read More

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http://dx.doi.org/10.3389/fphar.2018.00438DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5943597PMC
May 2018
4 Reads

Musculoskeletal Manifestations of Amyloidosis: A Focused Review.

J Surg Orthop Adv Spring 2018;27(1):1-5

Department of Orthopaedic Surgery, Howard University Hospital, Washington, D.C.

Amyloidosis is a poorly understood condition that can wreak havoc on numerous systems within the human body. In addition, this disease can present in multiple forms which each have their own unique physiology and subsequent effects. However, while the literature on the etiology and effect of amyloidosis on various organ systems is numerous, few have highlighted the musculoskeletal manifestations of this devastating disease. Read More

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July 2018
2 Reads

Severe white matter astrocytopathy in CADASIL.

Brain Pathol 2018 11;28(6):832-843

Neurovascular Research Group, Institute of Neuroscience, Newcastle University, Campus for Ageing & Vitality, Newcastle upon Tyne, UK.

Objectives: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is characterized by strategic white matter (WM) hyperintensities on MRI. Pathological features include WM degeneration, arteriolosclerosis, lacunar infarcts, and the deposition of granular osmiophilic material. Based on the hypothesis that the gliovascular unit is compromised, we assessed the nature of astrocyte damage in the deep WM of CADASIL subjects. Read More

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http://dx.doi.org/10.1111/bpa.12621DOI Listing
November 2018
28 Reads

Epidemiology of AL amyloidosis: a real-world study using US claims data.

Blood Adv 2018 05;2(10):1046-1053

Partnership for Health Analytic Research, LLC, Beverly Hills, CA; and.

Amyloid light-chain (AL) amyloidosis is a rare disease caused by extracellular deposition of misfolded immunoglobulin light chains. This study aimed to provide an up-to-date estimate of prevalence and incidence of AL amyloidosis in the United States. Using claims databases from years 2007 to 2015, adults ≥18 years old with AL amyloidosis were included if they had (1) at least 1 inpatient or 2 outpatient claims consistent with AL amyloidosis and (2) received 1 AL-specific treatment. Read More

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http://dx.doi.org/10.1182/bloodadvances.2018016402DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5965052PMC
May 2018
3 Reads

Wild-type transthyretin cardiac amyloidosis (ATTRwt-CA), previously known as senile cardiac amyloidosis: clinical presentation, diagnosis, management and emerging therapies.

J Thorac Dis 2018 Mar;10(3):2034-2045

Division of Cardiology, John Cochran Veterans Affairs Medical Center, St. Louis, USA.

Cardiac amyloidosis is thought to be a rare group of diseases caused by extracellular deposition of misfolded proteins in the extracellular cardiac matrix resulting in heart failure with preserved ejection fraction (HFpEF). This review focuses on the similarities and differences between the pathophysiology, clinical presentation and diagnostic tests of wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) compared to immunoglobulin light chain amyloidosis and hereditary cardiac amyloidosis. We address some obstacles to timely diagnosis and opportunities for management of the clinical symptoms as well as possibility of future novel disease modifying therapies. Read More

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http://dx.doi.org/10.21037/jtd.2018.03.134DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5906256PMC
March 2018
3 Reads

The Complexity and Heterogeneity of Monoclonal Immunoglobulin-Associated Renal Diseases.

J Am Soc Nephrol 2018 Jul 27;29(7):1810-1823. Epub 2018 Apr 27.

Department of Pathology, College of Physicians and Surgeons, Columbia University, New York, New York.

Monoclonal gammopathies are characterized by the overproduction of monoclonal Ig (MIg) detectable in the serum or urine resulting from a clonal proliferation of plasma cells or B lymphocytes. The underlying hematologic conditions range from malignant neoplasms of plasma cells or B lymphocytes, including multiple myeloma and B-cell lymphoproliferative disorders, to nonmalignant small clonal proliferations. The term MGUS implies presence of an MIg in the setting of a "benign" hematologic condition without renal or other end organ damage. Read More

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http://dx.doi.org/10.1681/ASN.2017121319DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6050917PMC
July 2018
3 Reads