1,747 results match your criteria Amyloidosis Primary Systemic


Primary Systemic Amyloidosis Mimicking Rhinophyma.

Indian Dermatol Online J 2019 Mar-Apr;10(2):204-205

Department of Dermatology, HBT Medical College and Cooper Hospital, Mumbai, Maharashtra, India.

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http://dx.doi.org/10.4103/idoj.IDOJ_163_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6434763PMC
April 2019
1 Read

Comparison of different techniques to identify cardiac involvement in immunoglobulin light chain (AL) amyloidosis.

Blood Adv 2019 Apr;3(8):1226-1229

Division of Hematology and.

We retrospectively reviewed the utility of N-terminal prohormone of brain natriuretic peptide (NT-proBNP) and transthoracic echocardiogram (TTE) in diagnosing cardiac involvement in patients with biopsy-proven systemic immunoglobulin light chain amyloidosis seen at the Mayo Clinic between 1 January 2006 and 30 December 2015. We analyzed 2 cohorts: patients undergoing endomyocardial biopsy for suspicion of cardiac involvement (cohort 1) and patients who had serum NT-proBNP and comprehensive echocardiographic evaluation at diagnosis (cohort 2). Of 179 patients undergoing endomyocardial biopsy (cohort 1), 173 (97%) had evidence of amyloid deposition, with 159 having NT-proBNP performed at the time of the procedure. Read More

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http://dx.doi.org/10.1182/bloodadvances.2019032458DOI Listing
April 2019
2 Reads

Primary brain amyloidoma, both a neoplastic and a neurodegenerative disease: a case report.

BMC Neurol 2019 Apr 10;19(1):59. Epub 2019 Apr 10.

Paul Flechsig Institute for Brain Research, University of Leipzig, Liebigstr. 19, 04103, Leipzig, Germany.

Background: Scattered extracellular deposits of amyloid within the brain parenchyma can be found in a heterogeneous group of diseases. Its condensed accumulation in the white matter without evidence for systemic amyloidosis is known as primary brain amyloidoma (PBA). Although originally considered as a tumor-like lesion by its space-occupying effect, this condition displays also common hallmarks of a neurodegenerative disorder. Read More

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http://dx.doi.org/10.1186/s12883-019-1274-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6458836PMC
April 2019
1 Read

Proteomics evidence of specific IGKV1-8 association with cystic lung light chain deposition disease.

Blood 2019 Apr 9. Epub 2019 Apr 9.

pathology, Institut Universitaire du Cancer (IUCT), France

We previously reported a new form of LCDD presenting as diffuse cystic lung disorder that differs from the usual systemic form, with respect to the age, the male/female ratio, the involved organs, and the hematologic characteristics. We also demonstrated that the light chains were produced by an intrapulmonary B-cell clone and, that this clone shared a stereotyped antigen receptor IGHV4-34/IGKV1. However, we analyzed only 3 patients. Read More

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http://dx.doi.org/10.1182/blood.2019898577DOI Listing
April 2019
2 Reads

Early Detection of Multiorgan Light Chain (AL) Amyloidosis by Whole Body F-florbetapir PET/CT.

J Nucl Med 2019 Apr 6. Epub 2019 Apr 6.

Brigham and Women's Hospital, United States.

Immunoglobulin light chain amyloidosis (AL) affects multiple systemic organs. However, determination of the precise extent of organ involvement remains challenging. Targeted amyloid imaging with F-florbetapir PET/CT offers the potential to detect AL amyloid deposits in multiple organs. Read More

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http://dx.doi.org/10.2967/jnumed.118.221770DOI Listing
April 2019
3 Reads

Mucocutaneous manifestations in systemic amyloidosis A retrospective analytical study in a tertiary care center.

Int J Dermatol 2019 Apr 2. Epub 2019 Apr 2.

Department of Dermatology, Instituto Nacional de Ciencias Médicas y Nutrición "Salvador Zubirán" (INCMNSZ), Mexico City, Mexico.

Background: The mucocutaneous clinical profile of patients with amyloidosis in Latin America has been scarcely reported.

Objective: To describe clinical characteristics of skin and mucosal manifestations in systemic amyloidosis in a tertiary care center in Mexico City.

Methods: A cross sectional, retrospective analytical study was performed in patients with systemic amyloidosis over a 15-year period. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1111/ijd.14443
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http://dx.doi.org/10.1111/ijd.14443DOI Listing
April 2019
5 Reads

Cryo-EM structure of a light chain-derived amyloid fibril from a patient with systemic AL amyloidosis.

Nat Commun 2019 03 20;10(1):1103. Epub 2019 Mar 20.

Institute of Protein Biochemistry, Ulm University, 89081, Ulm, Germany.

Amyloid fibrils derived from antibody light chains are key pathogenic agents in systemic AL amyloidosis. They can be deposited in multiple organs but cardiac amyloid is the major risk factor of mortality. Here we report the structure of a λ1 AL amyloid fibril from an explanted human heart at a resolution of 3. Read More

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http://dx.doi.org/10.1038/s41467-019-09032-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6427026PMC
March 2019
2 Reads

Cryo-EM structure of cardiac amyloid fibrils from an immunoglobulin light chain AL amyloidosis patient.

Nat Commun 2019 03 20;10(1):1269. Epub 2019 Mar 20.

Dipartimento di Bioscienze, Università degli Studi di Milano, Via Celoria 26, 20133, Milano, Italy.

Systemic light chain amyloidosis (AL)  is a life-threatening disease caused by aggregation and deposition of monoclonal immunoglobulin light chains (LC) in target organs. Severity of heart involvement is the most important factor determining prognosis. Here, we report the 4. Read More

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http://dx.doi.org/10.1038/s41467-019-09133-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6427027PMC
March 2019
2 Reads

A Patient with Sjogren's Syndrome and Subsequent Diagnosis of Inclusion Body Myositis and Light-Chain Amyloidosis.

J Gen Intern Med 2019 Mar 18. Epub 2019 Mar 18.

Stanford Center for Undiagnosed Diseases, Stanford University School of Medicine, Stanford, CA, USA.

We discuss a challenging case of a 58-year-old Vietnamese-American woman who presented to her new primary care provider with an 8-year history of slowly progressive dysphagia, hoarseness, muscle weakness with associated frequent falls, and weight loss. She eventually reported dry eyes and dry mouth, and she was diagnosed with Sjogren's syndrome. Subsequently, she was additionally diagnosed with inclusion body myositis and gastric light-chain (AL) amyloidosis. Read More

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http://dx.doi.org/10.1007/s11606-019-04931-wDOI Listing
March 2019
3 Reads

Acquired factor X deficiency in light-chain (AL) amyloidosis is rare and associated with advanced disease.

Hematol Oncol Stem Cell Ther 2019 Mar 28;12(1):10-14. Epub 2018 Jun 28.

Division of Hematology/Oncology, Department of Medicine, Medical College of Wisconsin, Milwaukee, WI, USA. Electronic address:

Introduction: Systemic light chain (AL) amyloidosis can lead to an acquired coagulopathy secondary to acquired factor X (aFX) deficiency. However, it is not very clear who develops aFX deficiency in AL amyloidosis.

Methods: We therefore undertook this single centre, retrospective study to better characterize AL amyloidosis-associated aFX deficiency. Read More

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http://dx.doi.org/10.1016/j.hemonc.2018.05.002DOI Listing
March 2019
3 Reads

AL-Amyloidoma of the Skin-A Rare Manifestation of Primary Cutaneous Marginal Zone Lymphoma.

Am J Dermatopathol 2019 Feb 25. Epub 2019 Feb 25.

Department of Dermatology, Venereology and Allergology, University Medical Centre, Göttingen, Germany.

AL-amyloidoma is considered to be a variant of primary cutaneous marginal zone lymphoma (pcMZL). A 51-year-old white man presented a 2 × 2-cm erythematous to brownish waxy plaque on the back of the scalp. The plaque was first noticed 16 years ago. Read More

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http://dx.doi.org/10.1097/DAD.0000000000001368DOI Listing
February 2019
2 Reads

Amyloid-Associated Alopecia: A Reappraisal Including Its Pathophysiology.

Am J Dermatopathol 2019 Feb 28. Epub 2019 Feb 28.

Department of Pathology and Laboratory Medicine, Dartmouth Hitchcock Medical Center, Lebanon, NH.

Primary systemic amyloidosis has a varied clinical presentation, making it one of the great masqueraders of other disease entities in clinical medicine. The association of amyloidosis with alopecia is uncommon with at least 22 cases reported in the literature mostly in the setting of systemic amyloidosis. Alopecia in these patients occurs either as the initial presentation of the systemic amyloidosis or it happens during the disease course. Read More

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http://dx.doi.org/10.1097/DAD.0000000000001385DOI Listing
February 2019
3 Reads

Classification of Plasma Cell Disorders by 21 Tesla Fourier Transform Ion Cyclotron Resonance Top-Down and Middle-Down MS/MS Analysis of Monoclonal Immunoglobulin Light Chains in Human Serum.

Anal Chem 2019 Mar 25;91(5):3263-3269. Epub 2019 Feb 25.

Department of Chemistry and Biochemistry , Florida State University , Tallahassee , Florida 32310 , United States.

The current five-year survival rate for systemic AL amyloidosis or multiple myeloma is ∼51%, indicating the urgent need for better diagnosis methods and treatment plans. Here, we describe highly specific and sensitive top-down and middle-down MS/MS methods owning the advantages of fast sample preparation, ultrahigh mass accuracy, and extensive residue cleavages with 21 telsa FT-ICR MS/MS. Unlike genomic testing, which requires bone marrow aspiration and may fail to identify all monoclonal immunoglobulins produced by the body, the present method requires only a blood draw. Read More

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http://dx.doi.org/10.1021/acs.analchem.8b03294DOI Listing
March 2019
6 Reads

Primary Systemic Amyloidosis.

Indian Dermatol Online J 2019 Jan-Feb;10(1):87-88

Department of Ophthalmology, Dayanand Medical College and Hospital, Ludhiana, Punjab, India.

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http://dx.doi.org/10.4103/idoj.IDOJ_175_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6362735PMC
February 2019

Cyclophosphamide + Thalidomide + Dexamethasone Versus Melphalan + Dexamethasone for the Treatment of Amyloid Light-chain Amyloidosis With Kidney Involvement: A Retrospective Study in Chinese Patients.

Clin Ther 2019 Feb 1. Epub 2019 Feb 1.

Department of Nephrology, Xijing Hospital, Air Force Medical University, Xi'an, China. Electronic address:

Purpose: At present, a diverse array of treatment regimens are available for systemic amyloid light-chain (AL) amyloidosis. Both cyclophosphamide + thalidomide + dexamethasone (CTD) and melphalan + dexamethasone (MD) regimens have been recommended as first-line therapies, but no detailed comparative studies of the two have been performed. This study is the first to compare the efficacy and tolerability of the CTD and MD regimens in the treatment of AL amyloidosis. Read More

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http://dx.doi.org/10.1016/j.clinthera.2018.12.003DOI Listing
February 2019
11 Reads

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
4 Reads

AL amyloidosis with a localized B cell neoplasia.

Virchows Arch 2019 Mar 24;474(3):353-363. Epub 2019 Jan 24.

Department of Pathology, Christian-Albrechts-University Kiel, Arnold-Heller-Str. 3/14, D-24105, Kiel, Germany.

Immunoglobulin light chain-derived (AL) amyloidosis may occur as a systemic disease usually with dismal prognosis and a localized variant with favorable outcome. We report 29 patients with AL amyloidosis and associated lymphoplasmacytic infiltrate spatially related to amyloid deposits. In 17 cases, the amyloid deposits were classified as ALλ and 12 as ALκ Histopathology in all cases showed relatively sparse plasma cells and B cells without tumor or sheet formation by the lymphoplasmacytic infiltrate. Read More

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http://dx.doi.org/10.1007/s00428-019-02527-7DOI Listing
March 2019
4 Reads

Nodular amyloidosis in a patient with systemic scleroderma.

Dermatol Online J 2018 Sep 15;24(9). Epub 2018 Sep 15.

Duke University Medical Center, Department of Dermatology, Durham, North Carolina.

Primary cutaneous amyloidosis may be characterized as macular amyloidosis, lichenoid amyloidosis, or nodular amyloidosis. Nodular amyloidosis results from the deposition of immunoglobulin light chains and may rarely be associated with systemic amyloidosis. We report an unusual case of a patient with systemic scleroderma who developed primary cutaneous nodular amyloidosis on the left lower leg. Read More

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September 2018
4 Reads

Light chain cardiac amyloidosis - a rare cause of heart failure in a young adult.

Rev Assoc Med Bras (1992) 2018 Sep;64(9):787-790

. Department of Cardiology, Hospital Distrital de Santarém, Santarém, Portugal.

Cardiac amyloidosis is an infiltrative cardiomyopathy, resulting from amyloid deposition within the myocardium. In primary systemic (AL-type) amyloidosis, the amyloid protein is composed of light chains resulting from plasma-cell dyscrasia, and cardiac involvement occurs in up to 50% of the patients We present a case of a 43-year-old man, with complaints of periodical swollen tongue and xerostomia, bleeding gums and haematuria for two months. His blood results showed normocytic anaemia, thrombocytopenia and a high spontaneous INR, therefore he was referred to the Internal Medicine clinic. Read More

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http://dx.doi.org/10.1590/1806-9282.64.09.787DOI Listing
September 2018
5 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
13 Reads

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

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

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

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
2 Reads

Case report: systemic tuberculosis caused by Mycobacterium bovis in a cat.

BMC Vet Res 2019 Jan 5;15(1). Epub 2019 Jan 5.

Department of Virology, Faculty of Veterinary Medicine, Ataturk University, Erzurum, Turkey.

Background: The diagnosis of previous cases of feline tuberculosis in Turkey has been made based solely on pathological changes without isolation of the causative agent. This case report details the first case of feline tuberculosis in Turkey for which the causative agent (Mycobacterium bovis) was confirmed with microbiological isolation, morphological evaluation, molecular (PCR) characterization and antibiotic sensitivity.

Case Presentation: Systemic tuberculosis was diagnosed via postmortem examination of a 5-year-old stray male cat. Read More

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http://dx.doi.org/10.1186/s12917-018-1759-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6321719PMC
January 2019
6 Reads

Histopathologic Evaluation of Flexor Tenosynovium in Recurrent Carpal Tunnel Syndrome.

Plast Reconstr Surg 2019 01;143(1):169-175

Phoenix, Ariz. From the Departments of Orthopedic Surgery and Laboratory Medicine and Pathology, Mayo Clinic Hospital.

Background: The authors' purpose was to evaluate the histopathology of flexor tenosynovium in true, idiopathic recurrent carpal tunnel syndrome for the presence of abnormal inflammatory or pathologic findings that might explain causation or that differ from those previously described for primary, idiopathic carpal tunnel syndrome.

Methods: Thirty-five patients (19 women and 16 men; mean age, 72 years) underwent open revision carpal tunnel release a mean 13 years (range, 0.5 to 30 years) after primary carpal tunnel release. Read More

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http://dx.doi.org/10.1097/PRS.0000000000005090DOI Listing
January 2019
17 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
4 Reads

Unusual morphology of amyloid cast nephropathy in renal biopsy portending poor prognosis.

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

Department of Nephrology, Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, India.

Light chain cast nephropathy is the most frequent form of renal disease in plasma cell neoplasm showing precipitation of monoclonal immunoglobulin light chains in the lumen of the distal tubules. This has a typical morphological feature characterised by the presence of a fractured cast. In this article, we report an unusual case of light chain cast nephropathy exhibiting amyloidogenic potential with lamellated, spiculated appearance. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22589
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http://dx.doi.org/10.1136/bcr-2018-225899DOI Listing
December 2018
14 Reads

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
2 Reads

Decompensated Liver Disease due to Primary Hepatic Amyloidosis: Is Liver Transplantation Still Mandatory?

Hepatology 2018 Dec 18. Epub 2018 Dec 18.

Systemic Autoimmune Diseases Unit, Internal Medicine Department, Hospital Universitario Vall d'Hebron, Barcelona, Spain.

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http://dx.doi.org/10.1002/hep.30464DOI Listing
December 2018
4 Reads

Parotid abscess secondary to brucellosis in a patient with primary Sjögren's syndrome.

Eur J Rheumatol 2018 Mar;5(1):62-64

Department of Rheumatology, Fırat University School of Medicine, Elazığ, Turkey.

Swelling in the salivary glands, particularly parotid glands, is observed in approximately one third of Sjögren's syndrome patients. However, such patients should be assessed in terms of causes such as malignancies, infections, amyloidosis, sarcoidosis, and other autoimmune diseases when parotid gland swelling is noted. It should be considered that the incidence of lymphoma increases in Sjögren's syndrome. Read More

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http://dx.doi.org/10.5152/eurjrheum.2017.16063DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6063305PMC
March 2018
2 Reads

Thermoregulation in neuropathies.

Authors:
Robert D Fealey

Handb Clin Neurol 2018 ;157:777-787

Department of Neurology, Mayo Clinic, Rochester, MN, United States. Electronic address:

Peripheral neuropathy affecting autonomic and small sensory fibers can cause abnormalities of both autonomic and behavioral thermoregulation. Quantitative autonomic and sensory neurophysiologic tests and quantification of the linear density of intraepidermal nerve fibers potentially can stratify those at risk of impaired thermoregulation during cold and heat challenges. New data relating to thermoregulatory sweating impairment in neuropathy are presented in this chapter. Read More

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http://dx.doi.org/10.1016/B978-0-444-64074-1.00048-3DOI Listing
March 2019
23 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
2 Reads

Suspected Pericardial Tuberculosis Revealed as an Amyloid Pericardial Mass.

Case Rep Hematol 2018 17;2018:8606430. Epub 2018 Oct 17.

Department of Biomedical Sciences and Human Oncology, Section of Internal Medicine "G. Baccelli", University of Bari Aldo Moro Medical School, Bari, Italy.

Primary systemic amyloidosis is not easily diagnosed. The immunoglobulin deposits are usually localized in the kidney, heart, and liver. We describe an unusual case of a patient suffering from a pericardial amyloidoma with internal calcifications and air bubbles that compressed the right ventricle and shifted the heart to the left. Read More

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https://www.hindawi.com/journals/crihem/2018/8606430/
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http://dx.doi.org/10.1155/2018/8606430DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6207883PMC
October 2018
20 Reads

A rare case of primary thyroid amyloidosis.

Int J Surg Case Rep 2018 25;53:179-181. Epub 2018 Oct 25.

Department of Medical and Surgical Sciences and Advanced Technologies, G.F. Ingrassia, Catania, Italy.

Introduction: Amyloid goiter is due to the deposition of amyloid in the thyroid, resulting with enlargement of the gland and compressive symptoms.

Case: We herein present a case of a 45-year-old male patient who complained of a big swelling in the neck. Ultrasound showed an enlarged thyroid gland with mediastinal involvement. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22102612183045
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http://dx.doi.org/10.1016/j.ijscr.2018.10.033DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6222088PMC
October 2018
14 Reads

Systemic immunoglobulin light chain amyloidosis.

Nat Rev Dis Primers 2018 Oct 25;4(1):38. Epub 2018 Oct 25.

Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, MN, USA.

Systemic immunoglobulin light chain amyloidosis is a protein misfolding disease caused by the conversion of immunoglobulin light chains from their soluble functional states into highly organized amyloid fibrillar aggregates that lead to organ dysfunction. The disease is progressive and, accordingly, early diagnosis is vital to prevent irreversible organ damage, of which cardiac damage and renal damage predominate. The development of novel sensitive biomarkers and imaging technologies for the detection and quantification of organ involvement and damage is facilitating earlier diagnosis and improved evaluation of the efficacy of new and existing therapies. Read More

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http://www.nature.com/articles/s41572-018-0034-3
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http://dx.doi.org/10.1038/s41572-018-0034-3DOI Listing
October 2018
25 Reads

Systemic immunoglobulin light chain amyloidosis.

Authors:

Nat Rev Dis Primers 2018 Oct 25;4(1):39. Epub 2018 Oct 25.

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http://dx.doi.org/10.1038/s41572-018-0040-5DOI Listing
October 2018
3 Reads

Identification of two principal amyloid-driving segments in variable domains of Ig light chains in systemic light-chain amyloidosis.

J Biol Chem 2018 12 24;293(51):19659-19671. Epub 2018 Oct 24.

From the Departments of Biological Chemistry and Chemistry and Biochemistry, Howard Hughes Medical Institute, UCLA-DOE Institute, UCLA, Los Angeles, California 90095 and

Systemic light-chain amyloidosis (AL) is a human disease caused by overexpression of monoclonal immunoglobulin light chains that form pathogenic amyloid fibrils. These amyloid fibrils deposit in tissues and cause organ failure. Proteins form amyloid fibrils when they partly or fully unfold and expose segments capable of stacking into β-sheets that pair and thereby form a tight, dehydrated interface. Read More

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http://www.jbc.org/lookup/doi/10.1074/jbc.RA118.004142
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http://dx.doi.org/10.1074/jbc.RA118.004142DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6314132PMC
December 2018
12 Reads

Intraoral manifestation of systemic AL amyloidosis with unique microscopic presentation of intracellular amyloid deposition in striated muscles.

Pol J Pathol 2018;69(2):200-204

We report the history of a 59-year old patient with systemic AL amyloidosis of intraoral manifestation. The patient first presented with complaints about dysphagia and remarkable enlargement of the tongue with highly reduced mobility, as well as bilateral submucosal thickenings on the cheeks. Histopathological examination of the incisional biopsy of the buccal mucosa and underlying tissues revealed AL amyloidosis. Read More

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https://www.termedia.pl/doi/10.5114/pjp.2018.76705
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http://dx.doi.org/10.5114/pjp.2018.76705DOI Listing
January 2018
19 Reads

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
March 2019
11 Reads

Dysphagia unveiling systemic immunoglobulin light-chain amyloidosis with multiple myeloma.

BMJ Case Rep 2018 Oct 21;2018. Epub 2018 Oct 21.

Internal Medicine, McLaren Health Care Corp, Flint, Michigan, USA.

Dysphagia is an uncommon presentation of systemic immunoglobulin light-chain (AL) amyloidosis with multiple myeloma (MM). Gastrointestinal (GI) involvement usually manifests with altered motility, malabsorption or bleeding. Furthermore, patients identified with GI amyloidosis, without previous diagnosis of a plasma cell disorder, are extremely rare. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22633
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http://dx.doi.org/10.1136/bcr-2018-226331DOI Listing
October 2018
12 Reads

Cytogenetic intraclonal heterogeneity of plasma cell dyscrasia in AL amyloidosis as compared with multiple myeloma.

Blood Adv 2018 Oct;2(20):2607-2618

Department of Medicine V, Hematology/Oncology/Rheumatology, University of Heidelberg, Heidelberg, Germany.

Analysis of intraclonal heterogeneity has yielded insights into the clonal evolution of hematologic malignancies. We compared the clonal and subclonal compositions of the underlying plasma cell dyscrasia in 544 systemic light chain amyloidosis (PC-AL) patients with 519 patients with monoclonal gammopathy of undetermined significance (MGUS), smoldering multiple myeloma (SMM), or symptomatic MM; ie, PC-non-AL patients). Using interphase fluorescence in situ hybridization, subclones were stringently defined as clone size below two thirds of the largest clone and an absolute difference of ≥30%. Read More

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http://www.bloodadvances.org/lookup/doi/10.1182/bloodadvance
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http://dx.doi.org/10.1182/bloodadvances.2018023200DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6199662PMC
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
20 Reads

Ameliorating effect of L. bark extract on histopathological changes following viper envenomation in animal models.

Toxicol Rep 2018 4;5:988-993. Epub 2018 Oct 4.

Department of Human Physiology with Community Health, Vidyasagar University, Paschim Medinipur-721102, West Bengal, India.

Objectives: The primary symptoms associated with snake envenomation are both systemic and local. The local symptoms are characterized by pain, swelling, haemorrhage and myonecrosis at the site of bite. The present study investigates the ameliorating effect of the aqueous bark extract of A bark on viper venom induced histopathological and biochemical changes in liver and kidney of swiss albino mice models. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22147500183021
Publisher Site
http://dx.doi.org/10.1016/j.toxrep.2018.10.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6180435PMC
October 2018
4 Reads

[Multifocal chalazodermic amyloidosis: The concept of immunoglobulinemic elastopathy].

Ann Dermatol Venereol 2018 Dec 9;145(12):777-784. Epub 2018 Oct 9.

Clinique dermatologique, université de Strasbourg, hôpitaux universitaires de Strasbourg, 1 place de l'hôpital, 67091, Strasbourg, France.

Introduction: Impairment of dermal elastic tissue occurs in different entities associated with immunoglobulins or immunoglobulin-derived protein-secreting clonal plasma cell proliferations, such as amyloid elastosis, anetodermic nodular amyloidosis or monoclonal gammopathy-associated cutis laxa. We report a case of cutaneous immunoglobulinemic amyloidosis revealed by a unique chalazodermic presentation and we review elastic tissue impairment in patients with monoclonal gammopathies.

Observation: A 67-year-old woman consulted for non-infiltrated anetodermic lesions on the upper left quadrant of her abdomen present for ten years. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01519638183052
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http://dx.doi.org/10.1016/j.annder.2018.07.021DOI Listing
December 2018
9 Reads

Primary Localized Cutaneous Amyloidosis Affecting Female Individuals of a Pakistani Pedigree.

Am J Dermatopathol 2019 May;41(5):382-385

Department of Dermatology, Chapel Allerton Hospital, Leeds, United Kingdom.

Primary localized cutaneous amyloidosis is a group of rare conditions where amyloid deposition is limited to the skin without systemic manifestations. Most cases are sporadic; however, mutations in the oncostatin M receptor (OSMR) and interleukin-31 receptor A (IL31RA) genes can cause a familial form of the condition in up to 10% of cases. Here, we describe a family in which 8 female individuals are affected by either macular amyloidosis or amyloidosis cutis dyschromica. Read More

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http://dx.doi.org/10.1097/DAD.0000000000001288DOI Listing
May 2019
6 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
5 Reads

Plasma ropivacaine concentration after TAP block in a patient with cardiac and renal failure.

Local Reg Anesth 2018 21;11:57-60. Epub 2018 Sep 21.

Department of Anesthesiology and Resuscitology, Shinshu University School of Medicine, Matsumoto, Nagano, Japan,

We report a successful ultrasound-guided transversus abdominis plane (TAP) block as an analgesic option for minor abdominal surgery in a 66-year-old patient with cardiac, respiratory, and renal dysfunction caused by primary systemic amyloidosis. Bilateral TAP blocks with 120 mg (1.8 mg/kg) of ropivacaine provided sufficient intra- and postoperative analgesia for insertion of a continuous ambulatory peritoneal dialysis catheter. Read More

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https://www.dovepress.com/plasma-ropivacaine-concentration-a
Publisher Site
http://dx.doi.org/10.2147/LRA.S173877DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6159791PMC
September 2018
11 Reads

Cutaneous light chain amyloidosis with multiple myeloma: A concise review.

Hematol Oncol Stem Cell Ther 2018 Sep 21. Epub 2018 Sep 21.

Division of Hematology & Oncology, Brookdale University Hospital Medical Center, Brooklyn, NY, USA. Electronic address:

Objective/background: Cutaneous immunoglobulin (Ig) amyloid light-chain (AL) amyloidosis associated with overt multiple myeloma (MM) is rare and optimal treatment is not well defined. The recently developed highly efficacious MM therapy has brought on a new set of challenges to this field for consideration. The goal of this paper is to describe the characteristics of cutaneous manifestations of systemic AL amyloidosis associated with MM according to age, sex, race, Ig type, plasma cell percentage, and cytogenetic and fluorescent in situ hybridization studies along with their outcomes. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S16583876183009
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http://dx.doi.org/10.1016/j.hemonc.2018.09.003DOI Listing
September 2018
76 Reads

IgM Myeloma: case report and literature review.

Ann Biol Clin (Paris) 2018 Oct;76(5):575-578

GCS de Saintonge, Centres hospitaliers de Saint-Jean-d'Angély, Jonzac, Saintes et Royan, Saint-Jean-d'Angély, France.

The presence of serum monoclonal IgM is often associated with the diagnosis of Waldenström macroglobulinemia (WM) or other chronic lymphoproliferative disorders. IgM myeloma is a rare entity (0.5%). Read More

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http://dx.doi.org/10.1684/abc.2018.1380DOI Listing
October 2018
7 Reads

Therapies for cardiac light chain amyloidosis: An update.

Int J Cardiol 2018 Nov;271:152-160

Amyloidosis Research and Treatment Centre, IRCCS Policlinico San Matteo, Pavia, Italy; Department of Molecular Medicine, University of Pavia, Italy.

Light-chain (AL) amyloidosis is the most common type of systemic amyloidosis, affecting around 10 people per million per year. This serious disorder is characterized by the presence of a clone of bone marrow plasma cells that produces monoclonal light chains (LCs) of the κ or predominantly λ type. These amyloidogenic LCs undergo extracellular misfolding and aggregation into proteotoxic soluble oligomers and amyloid fibrils that deposit within tissues. Read More

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http://dx.doi.org/10.1016/j.ijcard.2018.05.018DOI Listing
November 2018
3 Reads

Local vs. systemic pulmonary amyloidosis-impact on diagnostics and clinical management.

Virchows Arch 2018 Nov 22;473(5):627-637. Epub 2018 Aug 22.

Department of Pathology, Christian-Albrechts-University, Arnold-Heller-Str. 3/14, D-24105, Kiel, Germany.

Immunoglobulin-derived light-chain (AL) amyloidosis of lungs and bronchi can appear as a systemic and a local form. While systemic AL amyloidosis may need haemato-oncological care, the localised form can be treated restrained. We re-evaluated 207 specimens of lungs and bronchi sent in for amyloid diagnostics. Read More

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http://dx.doi.org/10.1007/s00428-018-2442-xDOI Listing
November 2018
29 Reads