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    1660 results match your criteria Amyloidosis Primary Systemic

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    Fibrillary Glomerulonephritis in a Patient with Sjogren's Syndrome.
    Cureus 2018 Apr 15;10(4):e2483. Epub 2018 Apr 15.
    Assistant Clinical Professor of Internal Medicine, West Virginia University School of Medicine.
    Fibrillary glomerulonephritis (FGN) is an uncommon cause of primary glomerular disease. FGN is usually idiopathic; however, it has been associated with underlying malignancy or autoimmune diseases in some patients as well. The most commonly found autoimmune diseases in FGN patients include Graves' disease, systemic lupus nephritis, Chron's disease, and idiopathic thrombocytopenia purpura. Read More

    Gastrointestinal Polyps and Hemorrhage as a Presentation of Primary Systemic Light Chain Amyloidosis.
    ACG Case Rep J 2018 6;5:e44. Epub 2018 Jun 6.
    Department of Gastroenterology, Texas Tech University Health Sciences Center, Lubbock, Texas.
    Light-chain amyloidosis is caused by deposition of immunoglobulin light chains within multiple organs, including the gastrointestinal (GI) tract. Gastrointestinal hemorrhage is a less frequent presentation. Endoscopic findings are nonspecific, and bleeding mucosal polyps are rare. Read More

    Primary solitary retro-clival amyloidoma.
    Surg Neurol Int 2018 15;9:100. Epub 2018 May 15.
    Department of Neurosurgery, Zucker School of Medicine at Hofstra/Northwell, Manhasset, New York, USA.
    Background: Amyloidosis encompasses a group of disorders sharing the common feature of intercellular deposition of amyloid protein by several different pathogenetic mechanisms. Primary solitary amyloidosis, or amyloidoma, is a rare subset of amyloidosis in which amyloid deposition is focal and not secondary to a systemic process or plasma cell dyscrasia.

    Case Description: This 84-year-old female presented with history of multiple syncopal episodes, dysphagia, and ataxia. Read More

    Ocular Adnexal Amyloidosis: A Mass Spectrometric Analysis.
    Am J Ophthalmol 2018 Jun 8. Epub 2018 Jun 8.
    Cole Eye Institute, Cleveland Clinic, 9500 Euclid Avenue, Cleveland, OH, 44195. Electronic address:
    Purpose: Ocular adnexal amyloidosis may represent localized manifestation of an underlying systemic process. Accurate identification of the amyloid fibrils can guide the systemic work up and treatment. The aim of this study was to characterize subtypes of ocular adnexal amyloidosis using immunohistochemistry and mass spectrometric analysis and to correlate with ocular involvement and systemic association. Read More

    Dysphagia due to macroglossia in a patient with amyloidosis associated with multiple myeloma: A case report.
    Spec Care Dentist 2018 Jun 11. Epub 2018 Jun 11.
    Program of Research in Health, Centro Universitário Cesmac, Maceió, AL, Brazil.
    Amyloidosis is a group of heterogeneous conditions characterized by the deposition of extracellular proteinaceous substances called amyloids. The diagnosis is usually confirmed after a particular body organ has been affected. However, oral manifestations have been identified in 39% of affected patients. Read More

    Management of the elderly patient with AL amyloidosis.
    Eur J Intern Med 2018 May 22. Epub 2018 May 22.
    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

    Cutaneous Features and Diagnosis of Primary Sjogren's Syndrome: An Update and Review.
    J Am Acad Dermatol 2018 May 19. Epub 2018 May 19.
    UConn Health, Department of Dermatology, Farmington, CT. Electronic address:
    Sjogren's syndrome (SS) is an autoimmune connective tissue disorder (CTD) which principally affects the lacrimal and salivary glands. Although SS is one of the three most common autoimmune CTDs alongside systemic lupus erythematosus (SLE) and progressive systemic sclerosis, it is the least researched CTD overall. SS poses a particular diagnostic challenge because it shares multiple clinical and immunologic features with other CTDs. Read More

    Canakinumab for the Treatment of Autoinflammatory Recurrent Fever Syndromes.
    N Engl J Med 2018 05;378(20):1908-1919
    From the Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, Rome (F.D.B.), Clinica Pediatrica e Reumatologia, Unità Operativa Semplice Dipartimentale di Malattie Autoinfiammatorie e Immunodeficienze, IRCCS, Istituto G. Gaslini, Genoa (M.G.), the Pediatric Clinic, University of Brescia and Spedali Civili, Brescia (M.C.), and the Amyloidosis Research and Treatment Center, Biotechnology Research Laboratories, Fondazione IRCCS Policlinico San Matteo, Pavia (L.O.) - all in Italy; the Division of Pediatric Rheumatology, Hospital Sant Joan de Déu, Universitat de Barcelona (J.A.), and the Internal Medicine Department, Autoimmune and Systemic Diseases Unit, Hospital Vall d'Hebron (S.B.-R.), Barcelona, and the Pediatric Rheumatology Unit, Hospital Universitario y Politécnico La Fe, Valencia (I.C.P.) - all in Spain; the Rheumatology Unit, Hadassah-Hebrew University Hospital (E.B.-C.), and the Pediatric Rheumatology Unit, Shaare Zedek Medical Center (P.J.H.), Jerusalem, and Heller Institute of Medical Research and Medicine Faculty, Sheba Medical Center, Tel-Hashomer and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv (A.L.) - all in Israel; the Division of Pediatrics, University Medical Center Utrecht, Utrecht (J.F.), and the Radboud Expertise Center for Immunodeficiency and Autoinflammation, Department of Internal Medicine, Radboud University Medical Center, Nijmegen (A. Simon) - both in the Netherlands; the Departments of Pediatrics and Medicine, University of California at San Diego and Rady Children's Hospital San Diego, San Diego (H.M.H.); the Department of Pediatric Rheumatology, Centre de Référence des Maladies Auto-inflammatoires et de l'Amylose Inflammatoire, Centre Hospitalier Universitaire (CHU) de Bicêtre, Assistance Publique-Hopitaux de Paris (APHP), Université de Paris Sud (I.K.-P.), and Paris-Descartes University, Imagine Institute, Unité d'Immuno-Hématologie et Rhumatologie Pédiatrique, Hôpital Necker-Enfants Malades, APHP (P.Q.), Paris; the National Amyloidosis Centre, University College London Division of Medicine, Royal Free Campus (H.J.L.), and University College London, Great Ormond Street Institute of Child Health, and Great Ormond Street Hospital for Children NHS Foundation Trust (P.B.), London; the Department of Pediatrics, Hacettepe University, Ankara (S.O.), and the Department of Pediatric Rheumatology, Cerrahpasa Medical School (O.K.), and Istanbul Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology (A.G.), Istanbul University, Istanbul - all in Turkey; the Department of Pediatrics, Division of Pediatric Rheumatology, Cleveland Clinic, Cleveland (A.Z.); the Department of Infectious Diseases and General Internal Medicine, CHU Sart-Tilman, University of Liège, Liege (M.M.), and the Department of Infectious Diseases and Immunity, Jessa Hospital, University of Hasselt, Hasselt (J.V.H.) - both in Belgium; the Department of Clinical Immunology, Center for Pediatric Hematology, Oncology, and Immunology, Moscow (A. Shcherbina); Pediatric Rheumatology of Western Switzerland, University of Lausanne, Lausanne, (M.H.), and Novartis, Basel (K.L., A. Speziale, G.J.) - both in Switzerland; the Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Japan (R.H.); and the Department of Pediatrics, Semmelweis Egyetem, Budapest, Hungary (T.C.).
    Background: Familial Mediterranean fever, mevalonate kinase deficiency (also known as the hyperimmunoglobulinemia D syndrome), and the tumor necrosis factor receptor-associated periodic syndrome (TRAPS) are monogenic autoinflammatory diseases characterized by recurrent fever flares.

    Methods: We randomly assigned patients with genetically confirmed colchicine-resistant familial Mediterranean fever, mevalonate kinase deficiency, or TRAPS at the time of a flare to receive 150 mg of canakinumab subcutaneously or placebo every 4 weeks. Patients who did not have a resolution of their flare received an add-on injection of 150 mg of canakinumab. Read More

    Primary endobronchial amyloidosis: A rare case of endobronchial tumor.
    Respir Med Case Rep 2018 26;23:163-166. Epub 2018 Feb 26.
    Pulmonary and Critical Care, Reliant Medical Group, Worcester MA, USA.
    Primary localized endobronchial amyloidosis is a rare entity, as pulmonary amyloidosis most commonly occurs as a part of systemic AL amyloidosis. It can be asymptomatic or can present with nonspecific symptoms such as progressive dyspnea, cough, wheezing and rarely respiratory failure. It is frequently misdiagnosed as asthma, COPD or pneumonia. Read More

    Cardiac amyloidosis.
    Clin Med (Lond) 2018 Apr;18(Suppl 2):s30-s35
    National Amyloidosis Centre, University College London, Royal Free Hospital, London, UK
    Systemic amyloidosis comprises an uncommon group of disorders caused by the extracellular deposition of misfolded proteins in various organs. Cardiac amyloid deposition, causing an infiltrative/restrictive cardiomyopathy, is a frequent feature of amyloidosis and a major determinant of survival. It may be the presenting feature of the disease or may be identified while investigating a patient presenting with other organ involvement. Read More

    Rationale and design of DUAL study: Doxycycline to Upgrade response in light chain (AL) amyloidosis (DUAL): A phase 2 pilot study of a two-pronged approach of prolonged doxycycline with plasma cell-directed therapy in the treatment of AL amyloidosis.
    Contemp Clin Trials Commun 2017 Dec 24;8:33-38. Epub 2017 Aug 24.
    Division of Hematology and Oncology, Medical College of Wisconsin, Milwaukee, WI 53226, United States.
    Light chain (AL) amyloidosis is a plasma cell neoplasm associated with insoluble fibril deposition from clonal immunoglobulin chains systemically. The disease is associated with high early mortality and morbidity owing to advanced organ deposition as well as lack of proven de-fibrillogenic therapies. Pre-clinical and retrospective clinical data suggests that doxycycline has benefit in AL amyloidosis. Read More

    Revisiting renal amyloidosis with clinicopathological characteristics, grading, and scoring: A single-institutional experience.
    J Lab Physicians 2018 Apr-Jun;10(2):226-231
    Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India.
    Introduction: Kidney involvement is a major cause of mortality in systemic amyloidosis. Glomerulus is the most common site of deposition in renal amyloidosis, and nephrotic syndrome is the most common presentation. Distinction between AA and AL is done using immunofluorescence (IF) and immunohistochemistry (IHC). Read More

    Plasma cell proliferative index predicts outcome in immunoglobulin light chain amyloidosis treated with stem cell transplantation.
    Haematologica 2018 Apr 19. Epub 2018 Apr 19.
    Division of Hematology, Department of Internal Medicine, Mayo Clinic Rochester;
    The plasma cell proliferative index provides an insight into plasma cell biology in plasma cell disorders and is an important prognostic marker in myeloma and smoldering myeloma. We analyzed the prognostic impact of the plasma cell proliferative index in 513 patients with systemic AL Amyloidosis undergoing stem cell transplantation at the Mayo Clinic between January 2003 and 31 August 2016. Two cohorts were identified; Low or Elevated plasma cell proliferative index. Read More

    Usefulness of gastroduodenal biopsy in the differential diagnosis of systemic AH amyloidosis from systemic AL amyloidosis.
    Histopathology 2018 Apr 16. Epub 2018 Apr 16.
    Department of Pathology, Shinshu University School of Medicine, Matsumoto, Japan.
    Aims: The aim of this study is to examine the usefulness of gastroduodenal biopsy for the detection of immunoglobulin (Ig) heavy-chain amyloid deposition. Ig heavy-chain amyloidosis (AH amyloidosis) is Ig-related amyloidosis classified together with Ig light-chain amyloidosis (AL amyloidosis). Compared with AL amyloidosis, patients with AH amyloidosis exhibit a better prognosis and they may not need an aggressive treatment. Read More

    Atrophic nodular cutaneous amyloidosis.
    An Bras Dermatol 2018 Jan-Feb;93(1):111-113
    Department of Dermatology, Jinling Hospital, Medical School of Nanjing University - Nanjing, China.
    Primary cutaneous amyloidosis is limited to the skin without involving any other tissue. Nodular amyloidosis is rare, and atrophic nodular cutaneous amyloidosis is even rarer. We describe the fourth case of atrophic nodular cutaneous amyloidosis by searching PubMed databases. Read More

    Pulmonary Nodular Amyloidosis Mimicking Metastatic Lung Lesion in a Patient with Colon Cancer.
    J Coll Physicians Surg Pak 2018 Apr;28(4):322-324
    Department of Surgery, College of Medicine, King Saud University, Riyadh, Kingdom of Saudi Arabia.
    Amyloidosis is defined as abnormal aggregation of amyloid proteins. Amyloidosis can be localised or systemic affecting the lung, heart, spleen, liver and kidneys. The most common form of pulmonary amyloidosis is the nodular type. Read More

    Primary systemic amyloidosis with skin and cardiac involvement: a case report
    Acta Dermatovenerol Alp Pannonica Adriat 2018 03;27(1):45-47
    Department of Dermatovenerology, University Medical Centre Maribor, Maribor, Slovenia.
    Primary systemic amyloidosis is characterized by the deposition of insoluble monoclonal immunoglobulin light chains in various tissues and is usually associated with an underlying plasma cell dyscrasia. In the early stage of the disease, dermatological findings can be the only manifestation, as opposed to organ involvement in the later stages. A dermatologist can diagnose amyloidosis early with a skin biopsy stained with Congo red dye and other appropriate investigations. Read More

    Small Bowel Amyloidosis.
    Curr Gastroenterol Rep 2018 Mar 26;20(3):11. Epub 2018 Mar 26.
    Division of Gastroenterology and Hepatology, Elmhurst Hospital Center, Icahn School of Medicine at Mount Sinai, Room D3-24D, 79-01 Broadway, Elmhurst, NY, 11373, USA.
    Purpose Of Review: The goal of this paper is to review the literature on small bowel amyloidosis. Our review focuses on the underlying etiology, histopathology, clinical features, endoscopic and radiologic findings, and the mainstay of management.

    Recent Findings: The latest research shows changing epidemiological trends of different types of amyloidosis. Read More

    Unilateral localized conjunctival amyloidosis in a patient with a history of contralateral orbit/eyelid lymphoma.
    Exp Mol Pathol 2018 Apr 15;104(2):151-154. Epub 2018 Mar 15.
    Department of Pathology and Laboratory Medicine, Harbor-UCLA Medical Center, Torrance, CA 90502, USA. Electronic address:
    Amyloidosis is a disorder characterized by the deposition of insoluble abnormal proteins in the extracellular space. It may occur as a localized lesion or as a systemic disease involving multiple organs and systems. Localized conjunctival amyloidosis is rare and is less frequently associated with systemic involvement. Read More

    [Treatment of systemic AL amyloidosis: about 25 cases].
    Pan Afr Med J 2017 19;28:160. Epub 2017 Oct 19.
    Service de Médecine Interne, Hôpital Militaire Moulay Ismail Méknes, Maroc.
    Primarye systemic AL amyloidosis is a rare hematologic disorder. The majority of the therapeutic guidelines are based on phase II studies or on retrospective comparisons and case series. Our study aimed to describe all the cases of primary AL amyloidosis reported in 2 military hospitals and to make a comparison between standard melphalan-dexamethasone protocol and new agents in first-line treatment of patients with this disease. Read More

    Truncation of ADAMTS13 by Plasmin Enhances Its Activity in Plasma.
    Thromb Haemost 2018 Mar 13;118(3):471-479. Epub 2018 Mar 13.
    Department for Clinical Chemistry and Haematology, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.
    ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) cleaves von Willebrand Factor (VWF) multimers to control their thrombogenicity. The fibrinolytic enzyme plasmin can cleave VWF in a similar manner. However, plasmin can also cleave ADAMTS13, which ultimately inactivates it. Read More

    Bone marrow plasma cell infiltration in light chain amyloidosis: impact on organ involvement and outcome.
    Amyloid 2018 Feb 26:1-7. Epub 2018 Feb 26.
    a Amyloidosis and Myeloma Unit, Department of Hematology , Hospital Clínic of Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona , Barcelona , Spain.
    Objectives: Prognosis of immunoglobulin light-chain (AL) amyloidosis depends mainly on the presence of cardiac involvement and the disease burden. A higher bone marrow plasma cell (BMPC) burden has been recognized as an adverse prognostic factor. The aim of our study was to analyze the correlation between the BMPC infiltration, clinical features and outcomes in patients with AL amyloidosis. Read More

    An unusual presentation of primary cutaneous amyloidosis.
    Dermatol Online J 2017 Aug 15;23(8). Epub 2017 Aug 15.
    Department of Dermatology and STD, Lady Hardinge Medical College. New Delhi, India.
    Primary localized cutaneous amyloidosis refers to a group of disorders characterized by deposition of amyloid in the dermis without any systemic involvement. It comprises the following clinical types: macular, lichenoid, nodular, and biphasic. There are also rare variants such as amyloidosis cutis dyscromica and poikiloderma-like cutaneous amyloidosis. Read More

    Oligosecretory Myeloma With Amyloidosis and Alopecia.
    J Investig Med High Impact Case Rep 2018 Jan-Dec;6:2324709617752737. Epub 2018 Jan 24.
    Albany Stratton VA Medical Center, Albany, NY, USA.
    Amyloidosis is a systemic illness characterized by the extracellular deposition of abnormal proteins in body tissues and organs. In addition to renal involvement, amyloidosis can also present with a variety of skin manifestations, though rarely with alopecia. Sixteen cases of alopecia secondary to systemic amyloidosis are reported. Read More

    Amyloid fibril polymorphism: a challenge for molecular imaging and therapy.
    J Intern Med 2018 Mar 19;283(3):218-237. Epub 2018 Feb 19.
    Department of Physics, Chemistry and Biology, Division of Chemistry, Linköping University, Linköping, Sweden.
    The accumulation of misfolded proteins (MPs), both unique and common, for different diseases is central for many chronic degenerative diseases. In certain patients, MP accumulation is systemic (e.g. Read More

    Unfolding Cardiac Amyloidosis - From Pathophysiology to Cure.
    Curr Med Chem 2018 Jan 4. Epub 2018 Jan 4.
    Division of Cardiology, Medical University of Graz, Graz. Austria.
    Deposition of amyloidogenic proteins leading to the formation of amyloid fibrils in the myocardium cause cardiac amyloidosis. Although any form of systemic amyloidosis can affect the heart, light-chain (AL) or transthyretin amyloidosis (ATTR) account for the majority of diagnosed cardiac amyloid deposition. The extent of cardiac disease independently predicts mortality. Read More

    Association of amyloidosis cutis dyschromica and familial Mediterranean fever.
    An Bras Dermatol 2017 ;92(5 Suppl 1):21-23
    Department of Gastroenterology, Mugla Sitki Kocman University Medical School - Mugla, Turkey.
    Amyloidosis cutis dyschromica is a rare type of primary cutaneous amyloidosis characterized by reticulate hyper-pigmentation with discrete hypopigmented macules. Up to date, about 50 cases of amyloidosis cutis dyschromica have been reported and the majority are familial cases of Asian ethnicity. Various diseases, particularly autoimmune diseases such as systemic sclerosis and systemic lupus erythematosus, have been associated with amyloidosis cutis dyschromica. Read More

    Structure and energetic basis of overrepresented λ light chain in systemic light chain amyloidosis patients.
    Biochim Biophys Acta 2018 Jun 12;1864(6 Pt B):2294-2303. Epub 2017 Dec 12.
    Basic Science Program, Leidos Biomedical Research, Inc., Cancer and Inflammation Program, National Cancer Institute, Frederick, MD 21702, USA. Electronic address:
    Amyloid formation and deposition of immunoglobulin light-chain proteins in systemic amyloidosis (AL) cause major organ failures. While the κ light-chain is dominant (λ/κ=1:2) in healthy individuals, λ is highly overrepresented (λ/κ=3:1) in AL patients. The structural basis of the amyloid formation and the sequence preference are unknown. Read More

    Recurrent diffuse gastric bleeding as a leading symptom of gastrointestinal AL amyloidosis.
    Z Gastroenterol 2017 Dec 6;55(12):1318-1322. Epub 2017 Dec 6.
    Klinik für Gastroenterologie, Hepatologie und Infektiologie, Universitätsklinikum Magdeburg, Germany.
    Amyloidosis is a rare disease (incidence about 0.8/100 000) characterized by extracellular tissue deposition of fibrils composed of low molecular weight subunits of a variety of serum proteins. Clinical manifestations are largely determined by the type of precursor protein, the tissue distribution and the amount of amyloid deposition. Read More

    Concurrent structural and biophysical traits link with immunoglobulin light chains amyloid propensity.
    Sci Rep 2017 Dec 1;7(1):16809. Epub 2017 Dec 1.
    Dipartimento di Bioscienze, Università degli Studi di Milano, 20133, Milano, Italy.
    Light chain amyloidosis (AL), the most common systemic amyloidosis, is caused by the overproduction and the aggregation of monoclonal immunoglobulin light chains (LC) in target organs. Due to genetic rearrangement and somatic hypermutation, virtually, each AL patient presents a different amyloidogenic LC. Because of such complexity, the fine molecular determinants of LC aggregation propensity and proteotoxicity are, to date, unclear; significantly, their decoding requires investigating large sets of cases. Read More

    Diffuse Peritoneal and Bowel Wall Infiltration by Light Chain-AL Amyloidosis with Omental Calcification Mimicking Abdominal Carcinomatosis - An Elderly Female with Incidental Finding of Light Chain Monoclonal Gammopathy of Undetermined Significance (LC-MGUS).
    Am J Case Rep 2017 Nov 25;18:1247-1250. Epub 2017 Nov 25.
    Icahn School of Medicine at Mount Sinai-Queens Hospital Center, Jamaica, NY, USA.
    BACKGROUND Amyloidosis is the extracellular tissue deposition of plasma proteins, which after conformational changes, forms antiparallel beta pleated sheets of fibrils. Amyloid light-chain (AL) is a type of amyloidosis that is due to deposition of proteins derived from immunoglobulin (Ig) light chains. Gastrointestinal tract (GIT) involvement most often found in amyloid A (AA) amyloidosis type. Read More

    Case for diagnosis. Systemic light chain amyloidosis with cutaneous involvement.
    An Bras Dermatol 2017 Sep-Oct;92(5):731-732
    Dermatology Clinic - Santa Casa de Belo Horizonte - Belo Horizonte (MG), Brazil.
    Systemic light chain amiloydosis is a rare disease. Due to its typical cutaneous lesions, dermatologists play an essential role in its diagnosis. Clinical manifestations vary according to the affected organ and are often unspecific. Read More

    Abdominal fat pad excisional biopsy for the diagnosis and typing of systemic amyloidosis.
    Hum Pathol 2018 Feb 11;72:71-79. Epub 2017 Nov 11.
    Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA. Electronic address:
    In the past, the diagnosis and typing of amyloidosis often required an invasive biopsy of an internal organ, such as the heart or kidneys. Abdominal fat pad excisional biopsy (FPEB) offers a less invasive approach, but the sensitivity of this technique has been unclear. To determine the sensitivity of FPEB for immunoglobulin light chain (AL) and transthyretin (ATTR) amyloidosis, we performed a retrospective clinicopathologic analysis of 97 patients who had undergone FPEB, of which 16 were positive for amyloid. Read More

    Regional differences in prognostic value of cardiac valve plane displacement in systemic light-chain amyloidosis.
    J Cardiovasc Magn Reson 2017 Nov 9;19(1):87. Epub 2017 Nov 9.
    Department of Cardiology, University of Heidelberg, INF 410, 69120, Heidelberg, Germany.
    Background: To compare the prognostic value of cardiac valve plane displacement (CVPD) on various locations in cardiac light chain (AL) amyloidosis.

    Methods: Consecutive patients with biopsy-proven cardiac involvement in AL amyloidosis who had undergone cardiovascular magnetic resonance (CMR) between 2005 and 2014 in our institution, were retrospectively identified and data analyzed. The primary combined endpoint was all-cause mortality or heart transplantation. Read More

    Elevation of Plasmin-α2-plasmin Inhibitor Complex Predicts the Diagnosis of Systemic AL Amyloidosis in Patients with Monoclonal Protein.
    Intern Med 2018 Mar 11;57(6):783-788. Epub 2017 Oct 11.
    Department of Gastroenterology, Rheumatology, and Clinical Immunology, Sapporo Medical University School of Medicine, Japan.
    Objective The complication of systemic immunoglobulin light chain (AL) amyloidosis in patients with monoclonal immunoglobulin affects the prognosis, but amyloid deposition in tissues is sometimes difficult to detect due to bleeding tendencies and preferential distributions. However, fibrinolysis is known to be exacerbated in patients with systemic AL amyloidosis specifically. We therefore explored new biomarkers for predicting a diagnosis of systemic AL amyloidosis focusing on coagulation and fibrinolysis markers. Read More

    Immunoparesis defined by heavy+light chain suppression is a novel marker of long-term outcomes in cardiac AL amyloidosis.
    Br J Haematol 2017 11 8;179(4):575-585. Epub 2017 Oct 8.
    Centre for Amyloidosis and Acute Phase Proteins, University College London (Royal Free Campus), London, UK.
    Cardiac involvement and presenting dFLC (difference between involved and uninvolved free light chains) are independent predictors of outcome in systemic AL amyloidosis. These markers have limited prognostic utility in patients surviving the initial months following diagnosis. Here we assessed immunoparesis, as determined by novel heavy+light chain (HLC) immunoassays, as a prognostic marker for survival in AL amyloidosis. Read More

    Idelalisib plus rituximab is effective in systemic AL amyloidosis secondary to chronic lymphocytic leukaemia.
    Hematol Oncol 2018 Feb 3;36(1):366-369. Epub 2017 Oct 3.
    Hematology and Clinical Immunology Unit, Department of Medicine, University of Padua, Padua, Italy.
    Light chain amyloidosis is characterized by the progressive deposition of immunoglobulin light chains into the extracellular tissue, leading to organ dysfunction. Usually, it is associated with an underlying clonal plasma cell dyscrasia and rarely with chronic lymphocytic leukaemia. Herein, we described the first report of a patient with relapsed chronic lymphocytic leukaemia harbouring TP53 abnormalities who developed, histologically proven, systemic light chain amyloidosis who was treated with the PI3K inhibitor, idelalisib, and rituximab. Read More

    Infectious and non-infectious complications in primary immunodeficiency disorders: an autopsy study from North India.
    J Clin Pathol 2018 May 28;71(5):425-435. Epub 2017 Sep 28.
    Department of Paediatrics (Allergy and Immunology Unit), Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India.
    Background: Primary immunodeficiency disorders (PID) include a wide spectrum of inherited disorders characterised by functional abnormalities of one or more components of the immune system. Recent updates from the genomic data have contributed significantly to its better understanding with identification of new entities. Diagnosis is always challenging due to their variable clinical presentation. Read More

    Current insight in the localized insulin-derived amyloidosis (LIDA): clinico-pathological characteristics and differential diagnosis.
    Pathol Res Pract 2017 Oct 1;213(10):1237-1241. Epub 2017 Sep 1.
    Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. Electronic address:
    Background: In diabetic patients, subcutaneous insulin injection may cause several types of injection site-related lesions, such as lipoatrophy, insulin-induced cutaneous lipohypertrophy (IICL), allergic reaction, and iatrogenic localized insulin-derived amyloidosis (LIDA). Among these complications, both IICL and LIDA present as tumor-like and slow growing lesions; and they may be confused with one another. The clinical implication and management of IICL and LIDA are different. Read More

    Utility of abdominal skin plus subcutaneous fat and rectal mucosal biopsy in the diagnosis of AL amyloidosis with renal involvement.
    PLoS One 2017 19;12(9):e0185078. Epub 2017 Sep 19.
    National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China.
    Objectives: Skin fat biopsy of the abdominal wall is a simple and safe method for detecting amyloidosis, and rectal mucosal biopsy is also frequently used for screening for the disease; however, the sensitivity of these approaches has not been fully studied. The aim of this study was to evaluate the efficacy of skin fat biopsy combined with rectal mucosal biopsy as a screening procedure for the diagnosis of systemic immunoglobulin light-chain (AL) amyloidosis.

    Methods: We retrospectively analyzed 224 AL amyloidosis patients confirmed by renal biopsy, including a test group of 165 patients and validation group of 59 patients. Read More

    The Lung in Dysregulated States of Humoral Immunity.
    Respiration 2017 15;94(5):389-404. Epub 2017 Sep 15.
    Université Paris 13, Sorbonne-Paris-Cité, EA2363, Paris, France.
    In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Read More

    Hepatic Amyloidosis: Something That Can camouflage and Deceive our Perception!
    Indian J Med Paediatr Oncol 2017 Apr-Jun;38(2):236-239
    Department of Gastroenterology, Kerala Institute of Medical Science and Research Centre, Trivandrum, Kerala, India.
    Amyloidosis is a multi-systemic diffusely infiltrating disease due to extracellular deposition of protein-mucopolysaccharide complexes. The type of protein deposited determines the subgroup of amyloid. Hepatic amyloidosis is a rare infiltrating disease affecting the hepatic parenchyma. Read More

    Visualization of multiple organ amyloid involvement in systemic amyloidosis using C-PiB PET imaging.
    Eur J Nucl Med Mol Imaging 2018 03 10;45(3):452-461. Epub 2017 Sep 10.
    Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano, 390-8621, Japan.
    Purpose: To investigate the utility of Pittsburgh compound B (PiB) positron emission tomography (PET) imaging for evaluating whole-body amyloid involvement in patients with systemic amyloidosis.

    Methods: Whole-body C-PiB PET was performed in seven patients with systemic immunoglobulin light-chain (AL) amyloidosis, seven patients with hereditary transthyretin (ATTRm) amyloidosis, one asymptomatic TTR mutation carrier and three healthy controls. The correlations between clinical organ involvement, radiological C-PiB uptake and histopathological findings were analysed for each organ. Read More

    Characterization of isolated amyloid myopathy.
    Eur J Neurol 2017 12 5;24(12):1437-1445. Epub 2017 Oct 5.
    Department of Neurology, Mayo Clinic, Rochester, MN, USA.
    Background And Purpose: Amyloid myopathy frequently occurs in the setting of systemic amyloidosis and less commonly in isolation (isolated amyloid myopathy). Anoctaminopathy-5 and dysferlinopathy were recently recognized as causes of isolated amyloid myopathy. The present study aimed to characterize the isolated amyloid myopathy and to compare it with amyloid myopathy associated with systemic amyloidosis. Read More

    Clinical characteristics of laryngeal versus nonlaryngeal amyloidosis.
    Laryngoscope 2018 Mar 4;128(3):670-674. Epub 2017 Sep 4.
    Department of Otolaryngology-Head and Neck Surgery, Stanford School of Medicine, Stanford University, Stanford, California, U.S.A.
    Objectives/hypothesis: Amyloidosis represents a heterogeneous group of disorders marked by abnormal protein formation and deposition. Laryngeal amyloidosis is rare and classically thought to remain isolated with little risk of systemic involvement or associated malignancy. This study sought to further characterize differences in clinical characteristics between patients with laryngeal and nonlaryngeal amyloidosis. Read More

    A Plasma Cell Dyscrasia Presenting as Amyloid Cardiomyopathy and Autonomic Dysfunction in a Healthy Patient.
    Cureus 2017 Jun 29;9(6):e1409. Epub 2017 Jun 29.
    Cardiology, Staten Island University Hospital, Northwell Health.
    Systemic amyloidosis is a rare multisystem disease caused by incorrectly folded proteins that deposit pathologically in different tissues and organs of the human body. It has a very wide spectrum of clinical presentations according to the affected organ(s), and its diagnosis is commonly delayed. Cardiac involvement is the leading cause of morbidity and mortality and carries a poor prognosis, especially in primary light chain amyloidosis. Read More

    [Amyloidoma Adjacent to the Thoracic Vertebral Body Developed into Systemic Amyloid Light-chain amyloidosis].
    Kyobu Geka 2017 Aug;70(9):755-757
    Department of Respiratory Medicine, Yokohama Medical Center, Yokohama, Japan.
    Amyloidoma is very rare case of amyloidosis, and seldom develop into systemic disease. We report a case of an 82-year-old man who was referred to our hospital because of an oppressive feeling in the upper chest. Chest computed tomography showed a tumor on thoracic vertebral body. Read More

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