1,052 results match your criteria Light-Chain Deposition Disease

Proliferative glomerulonephritis with monoclonal immunoglobulin G deposits in a young woman: A case report.

World J Clin Cases 2021 Apr;9(10):2357-2366

Department of Nephrology, Shenzhen Longhua District Central Hospital, Shenzhen 518110, Guangdong Province, China.

Background: Proliferative glomerulonephritis with monoclonal immunoglobulin G (IgG) deposits (PGNMID) is a newly recognized rare disease. The renal pathology is characterized by prominent manifestations of membranous hyperplasia, which are easy to misdiagnose. The clinical symptoms are severe. Read More

View Article and Full-Text PDF

Narrative review of pharmacotherapy for transthyretin cardiac amyloid.

Ann Transl Med 2021 Mar;9(6):519

Division of Cardiovascular Diseases, University of Alabama at Birmingham, Birmingham, AL, USA.

Treatment of cardiac amyloidosis is determined by the amyloid type and degree of involvement. Two types of amyloid commonly infiltrate the heart: immunoglobulin light-chain amyloid (AL), and transthyretin amyloid (ATTR), that encompasses other two forms, a hereditary form (hATTR), and a sporadic, age-related wild-type (wtATTR). The prevalence is expected to increase with aging population. Read More

View Article and Full-Text PDF

Monoclonal gammopathy of renal significance: Early diagnosis is key.

Nefrologia 2021 Apr 3. Epub 2021 Apr 3.

Nephrology Department, Carlos Haya Regional University Hospital and University of Malaga, IBIMA, REDinREN (RD16/0009/0006), Malaga, Spain. Electronic address:

Monoclonal gammopathy of renal significance is a clinical-pathological entity grouping renal disorders secondary to the secretion of a monoclonal immunoglobulin synthesized by a B-cell-derived clone and/or plasma cells in a patient with no diagnostic criteria for multiple myeloma. This term applies to a concept recently introduced owing to the need to differentiate this entity from monoclonal gammopathy of undetermined significance, given the negative prognostic impact of its high morbidity and mortality resulting from both renal and systemic involvement, occasionally even progressing to advanced chronic kidney disease. The renal damage occurs via both direct pathogenic mechanisms, with the deposition of the monoclonal protein in different renal structures, as well as indirect mechanisms, acting as an autoantibody provoking dysregulation of the alternative complement pathway. Read More

View Article and Full-Text PDF

Randall-Type Monoclonal Immunoglobulin Deposition Disease: New Insights into the Pathogenesis, Diagnosis and Management.

Diagnostics (Basel) 2021 Mar 2;11(3). Epub 2021 Mar 2.

Department of Nephrology, CHU Poitiers, 86000 Poitiers, France.

Randall-type monoclonal immunoglobulin deposition disease (MIDD) is a rare disease that belongs to the spectrum of monoclonal gammopathy of renal significance (MGRS). Renal involvement is prominent in MIDD, but extra-renal manifestations can be present and may affect global prognosis. Recent data highlighted the central role of molecular characteristics of nephrotoxic monoclonal immunoglobulins in the pathophysiology of MIDD, and the importance of serum free light chain monitoring in the diagnosis and follow-up disease. Read More

View Article and Full-Text PDF

Monoclonal and polyclonal immunoglobulin G deposits on tubular basement membranes of native and pretransplant kidneys: A retrospective study.

Pathol Int 2021 Mar 30. Epub 2021 Mar 30.

Department of Analytic Human Pathology, Nippon Medical School, Tokyo, Japan.

Monoclonal tubular basement membrane immune deposits (TBMID) are associated with progression of interstitial injury in renal allograft. However, the significance of monoclonal and polyclonal TBMID in the native kidney remains unclear. We retrospectively analyzed 1894 native kidney biopsies and 1724 zero-hour biopsies performed between 2008 and 2018 in our institution. Read More

View Article and Full-Text PDF

Case Report of a Rare Incidence of IgH Amplification Leading to Acute Kidney Injury in a Multiple Myeloma Patient.

Case Rep Oncol 2021 Jan-Apr;14(1):274-278. Epub 2021 Mar 2.

Tumor Immunology Program, Mazumdar Shaw Medical Foundation, Bangalore, India.

We present a case report of a 62-year-old male, treated for kappa light chain multiple myeloma with chemotherapy followed by autologous stem cell transplant (ASCT) in 2014. He has been in complete remission for 4 years. In 2018, he was evaluated for hypercreatinemia and acute kidney injury(AKI) with a suspicion of disease relapse; he underwent evaluation with bone marrow aspiration cytology which showed no evidence of relapse. Read More

View Article and Full-Text PDF

Immunofluorescence staining for immunoglobulin heavy chain/light chain on kidney biopsies is a valuable ancillary technique for the diagnosis of monoclonal gammopathy-associated kidney diseases.

Kidney Int 2021 Mar 25. Epub 2021 Mar 25.

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

Heavy chain/light chain (HLC) antibodies target conformational epitopes at the junctions of the heavy chain and light chain constant regions (CH1 and CL) of serum IgGκ, IgGλ, IgAκ, IgAλ, IgMκ, and IgMλ to provide quantitation of intact HLC pairs. Here, we developed an HLC tissue immunofluorescence protocol to test if it can complement conventional immunofluorescence in the diagnosis of monoclonal gammopathy-associated kidney diseases. HLC immunofluorescence was performed on archived frozen tissue of 104 kidney biopsies. Read More

View Article and Full-Text PDF

Proliferative Glomerulonephritis With Fibrils, Monoclonal κ Light Chain, and C3 Deposits.

Am J Kidney Dis 2021 Mar 24. Epub 2021 Mar 24.

Departments of Nephrology; Department of Medicine (RMH), University of Melbourne, Victoria, Australia.

There is increasing recognition of monoclonal gammopathy as a cause of proliferative glomerulonephritis (GN), including cases in which glomerular deposition of monoclonal immunoglobulin (mIg) is demonstrated. Recently, proliferative GN with mIg deposits (PGNMID) has incorporated a light chain (LC) variant of the disease (termed PGNMID-LC). Intriguingly, glomerular co-deposition of complement C3 is found in addition to monotypic LC, implying complement activation via the alternative pathway (AP). Read More

View Article and Full-Text PDF

Localized Peritumoral AL Amyloidosis Associated With Mantle Cell Lymphoma With Plasmacytic Differentiation.

Am J Surg Pathol 2021 Feb 2. Epub 2021 Feb 2.

*Hematopathology Service ‡Cytogenetic Service, Department of Pathology §Myeloma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, NY †Department of Pathology, Amsterdam UMC, location VU University Medical Center, Amsterdam, The Netherlands.

Immunoglobulin light chain (AL) amyloidosis is characterized by the deposition of amyloid fibers derived from pathologic immunoglobulin light chains. Although systemic plasma cell neoplasms are the most common cause of AL amyloidosis, a subset of cases is caused by B-cell lymphoproliferative disorders such as lymphoplasmacytic lymphoma or extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue. Recently, SOX11-negative IGH hypermutated mantle cell lymphoma (MCL) is recognized to show frequent plasmacytic differentiation and indolent clinical course. Read More

View Article and Full-Text PDF
February 2021

Amyloid cranial polyneuropathy: A rare neurological presentation of immunoglobulin light-chain amyloidosis.

Aging Med (Milton) 2021 Mar 5;4(1):61-65. Epub 2021 Feb 5.

Department of Geriatrics Christian Medical College and Hospital Vellore Vellore India.

Amyloidosis, a disease with extracellular tissue deposition of fibrils, results in clinical manifestations based on deposition of these fibrils in multiple organ systems. Usual manifestations include nephrotic-range proteinuria, cardiac failure, hepatosplenomegaly, and skin manifestations. Common neurological manifestations include peripheral and autonomic neuropathies. Read More

View Article and Full-Text PDF

The urine light chain/glomerular filtration rate (GFR) quotient shows a high sensitivity and specificity to detect cast nephropathy in monoclonal light chain disease.

Eur J Haematol 2021 Jun 24;106(6):836-841. Epub 2021 Mar 24.

Medizinische Klinik A, Klinikum der Stadt Ludwigshafen gGmbH, Ludwigshafen, Germany.

Background: Cast nephropathy (CN) is associated with a unfavourable outcome in monoclonal light chain (mLC) disease, but also more possible LC-related renal diseases as well as non-LC-related disease can occur. Thus, it is crucial to understand the underlying renal disease. On the other hand, LC can interfere with coagulation preventing kidney biopsy as the gold standard. Read More

View Article and Full-Text PDF

Pulmonary Vascular Disease Due to Plasma Cell Dyscrasia.

Mayo Clin Proc Innov Qual Outcomes 2021 Feb 20;5(1):210-218. Epub 2020 Nov 20.

Division of Cardiovascular Disease, Section of Advanced Heart Failure and Transplantation, University of Alabama at Birmingham, Birmingham.

Pulmonary hypertension (PH) has been described in myeloproliferative disorders; monoclonal plasma cell disorder such as polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome; and plasma cell dyscrasias such as multiple myeloma and amyloidosis. We describe 4 cases of PH likely due to pulmonary vascular involvement and myocardial deposition from light chain deposition disease, amyloidosis, and multiple myeloma. On the basis of our clinical experience and literature review, we propose screening for plasma cell dyscrasia in patients with heart failure with preserved ejection fraction, unexplained PH, and hematological abnormalities. Read More

View Article and Full-Text PDF
February 2021

A Case Report on Papillary Muscle Rupture Following Mitral Valve Replacement in the Setting of Previously Undiagnosed Amyloidosis.

A A Pract 2021 Mar 8;15(3):e01415. Epub 2021 Mar 8.

From the Department of Anesthesia, Critical Care and Pain Medicine.

This case describes a patient who underwent mitral valve replacement (MVR) surgery with preservation of the subvalvular apparatus who suffered anterolateral papillary muscle rupture (PMR) postseparation from cardiopulmonary bypass. This patient had no history of coronary artery disease (CAD); subsequent pathology of the papillary muscle showed evidence of amyloid deposition. Although most PMRs are caused by ischemia from CAD, cardiac amyloidosis must be considered in the absence of CAD and worked up appropriately as cardiac involvement of amyloidosis, especially the amyloid light-chain (AL) subtype, is prognostic of increased mortality that can be mitigated with therapy. Read More

View Article and Full-Text PDF

Crosstalk between MicroRNA and Oxidative Stress in Primary Open-Angle Glaucoma.

Int J Mol Sci 2021 Feb 28;22(5). Epub 2021 Feb 28.

Department of Clinical Biochemistry and Pharmacology, Ben-Gurion University of the Negev, Beer-Sheva 84105, Israel.

Reactive oxygen species (ROS) plays a key role in the pathogenesis of primary open-angle glaucoma (POAG), a chronic neurodegenerative disease that damages the trabecular meshwork (TM) cells, inducing apoptosis of the retinal ganglion cells (RGC), deteriorating the optic nerve head, and leading to blindness. Aqueous humor (AH) outflow resistance and intraocular pressure (IOP) elevation contribute to disease progression. Nevertheless, despite the existence of pharmacological and surgical treatments, there is room for the development of additional treatment approaches. Read More

View Article and Full-Text PDF
February 2021

Ghrelin protects against rotenone-induced cytotoxicity: Involvement of mitophagy and the AMPK/SIRT1/PGC1α pathway.

Neuropeptides 2021 Feb 19;87:102134. Epub 2021 Feb 19.

Neurobiology Institute, Jining Medical University, 272067 Jining, China. Electronic address:

Parkinson's disease (PD) is the second most common neurodegenerative disorder, characterized by the loss of dopaminergic neurons in the substantia nigra and the deposition of Lewy bodies. Mitochondrial dysfunction, oxidative stress, and autophagy dysfunction are involved in the pathogenesis of PD. Ghrelin is a brain-gut peptide that has been reported that protected against 1-methyl-4-phenyl-1,2,3,6- tetrahydropyran (MPTP)/MPP-induced toxic effects. Read More

View Article and Full-Text PDF
February 2021

Plasma cell neoplasms of the bladder: a series of 9 cases.

Hum Pathol 2021 Feb 24;111:52-58. Epub 2021 Feb 24.

Department of Urology, Johns Hopkins Medical Institutions, Baltimore, MD, USA; Department of Oncology, Johns Hopkins Medical Institutions, Baltimore, MD, USA. Electronic address:

From 2009 to 2017, we identified 9 cases of plasma cell neoplasms on biopsies of the bladder in patients without a history of plasma cell myeloma or transplantation (6 men and 3 women). Four of the nine showed amyloid deposition, of which one additionally revealed a clear cell adenocarcinoma of the bladder. Follow-up was obtained in 7 cases. Read More

View Article and Full-Text PDF
February 2021

A Unique Case of Combined Nodular and Tracheobronchial Amyloidosis.

Oxf Med Case Reports 2021 Feb 15;2021(2):omaa134. Epub 2021 Feb 15.

Department of Pulmonary and Critical Care Medicine, Cleveland Clinic, Cleveland, Ohio, USA.

Amyloidosis is a heterogeneous group of diseases characterized by the extracellular deposition of misfolded proteins that can affect either systemically or locally confined to one system. Pulmonary amyloidosis is rare and can be classified into three forms according to the anatomic site of involvement: nodular pulmonary amyloidosis, tracheobronchial amyloidosis and diffuse alveolar-septal amyloidosis. The former two usually represent localized amyloid disease and the latter represents systemic disease. Read More

View Article and Full-Text PDF
February 2021

Clinical approach to genetic testing in amyloid cardiomyopathy: from mechanism to effective therapies.

Curr Opin Cardiol 2021 05;36(3):309-317

Section of Cardiovascular Medicine, Department of Medicine.

Purpose Of Review: To highlight the evolving understanding of genetic variants, utility of genetic testing, and the selection of novel therapies for cardiac amyloidosis.

Recent Findings: The last decade has seen considerable progress in cardiac amyloidosis recognition given the advancement in cardiac imaging techniques and widespread availability of genetic testing. A significant shift in the understanding of a genetic basis for amyloidosis has led to the development of disease-modifying therapeutic strategies that improve survival. Read More

View Article and Full-Text PDF

Cardiac amyloidosis masquerading as acute coronary syndrome.

BMJ Case Rep 2021 Feb 18;14(2). Epub 2021 Feb 18.

Edinburgh Heart Centre, Royal Infirmary of Edinburgh, Edinburgh, UK.

A 53-year-old man presented to a district general hospital with chest pain, ECG changes and a small high-sensitivity cardiac troponin I rise. There were no symptoms of heart failure. CT coronary angiography revealed moderate calcific disease and conventional angiography confirmed no flow limitation. Read More

View Article and Full-Text PDF
February 2021

Amyloidosis, extramedullary plasmacytoma, and light chain deposition disease: impressive results of daratumumab therapy.

Pol Arch Intern Med 2021 03 11;131(3):297-298. Epub 2021 Feb 11.

Department of Hematology, Jagiellonian University Medical College, Kraków, Poland.

View Article and Full-Text PDF

Apolipoprotein E4 Reduction with Antisense Oligonucleotides Decreases Neurodegeneration in a Tauopathy Model.

Ann Neurol 2021 05 24;89(5):952-966. Epub 2021 Feb 24.

Department of Neurology, Hope Center for Neurological Disorders, Knight Alzheimer's Disease Research Center, Washington University School of Medicine, St. Louis, MO.

Objective: Apolipoprotein E (ApoE) genotype is the strongest genetic risk factor for late-onset Alzheimer's disease, with the ε4 allele increasing risk in a dose-dependent fashion. In addition to ApoE4 playing a crucial role in amyloid-β deposition, recent evidence suggests that it also plays an important role in tau pathology and tau-mediated neurodegeneration. It is not known, however, whether therapeutic reduction of ApoE4 would exert protective effects on tau-mediated neurodegeneration. Read More

View Article and Full-Text PDF

Two distinct mechanisms of neuropathy in immunoglobulin light chain (AL) amyloidosis.

J Neurol Sci 2021 Feb 2;421:117305. Epub 2021 Jan 2.

Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan. Electronic address:

Introduction: Although polyneuropathy in patients with immunoglobulin light chain (AL) amyloidosis has been considered to be attributable to axonal degeneration resulting from amyloid deposition, patients with nerve conduction parameters indicating demyelination that mimics chronic inflammatory demyelinating polyneuropathy (CIDP) have also been reported anecdotally.

Methods: We evaluated the electrophysiological and pathological features of 8 consecutive patients with AL amyloidosis who were referred for sural nerve biopsy.

Results: Although findings of axonal neuropathy predominantly in the lower limbs were the cardinal feature, all patients showed one or more abnormalities of nerve conduction velocities or distal motor latencies. Read More

View Article and Full-Text PDF
February 2021

Renal Expression of Light Chain Binding Proteins.

Front Med (Lausanne) 2020 13;7:609582. Epub 2021 Jan 13.

Department of Medicine III, Division of Nephrology and Dialysis, Medical University of Vienna, Vienna, Austria.

Overproduction of human light chains (LCs) and immunoglobulins can result in various forms of renal disease such as cast nephropathy, monoclonal immunoglobulin deposition disease, LC proximal tubulopathy, AL amyloidosis, and crystal storing histiocytosis. This is caused by cellular uptake of LCs and overwhelmed intracellular transport and degradation in patients with high urine LC concentrations. LC kappa and lambda purification was evaluated by sodium dodecyl sulfate gel electrophoresis. Read More

View Article and Full-Text PDF
January 2021

Molecular mechanism of amyloidogenic mutations in hypervariable regions of antibody light chains.

J Biol Chem 2021 Jan 25:100334. Epub 2021 Jan 25.

Center for Integrated Protein Science Munich at the Department Chemie, Technische Universität München, Lichtenbergstr. 4, 85748 Garching, Germany. Electronic address:

Systemic light chain (AL) amyloidosis is a fatal protein misfolding disease in which excessive secretion, misfolding, and subsequent aggregation of free antibody light chains eventually leads to deposition of amyloid plaques in various organs. Patient-specific mutations in the antibody V domain are closely linked to the disease, but the molecular mechanisms by which certain mutations induce misfolding and amyloid aggregation of antibody domains are still poorly understood. Here, we compare a patient V domain with its non-amyloidogenic germline counterpart and show that, out of the five mutations present, two of them strongly destabilize the protein and induce amyloid fibril formation. Read More

View Article and Full-Text PDF
January 2021

New Insights into the Role of Ferritin in Iron Homeostasis and Neurodegenerative Diseases.

Mol Neurobiol 2021 Jan 28. Epub 2021 Jan 28.

Department of Physiology, Shandong Provincial Key Laboratory of Pathogenesis and Prevention of Neurological Disorders, School of Basic Medicine, Qingdao University, Qingdao, 266071, China.

Growing evidence has indicated that iron deposition is one of the key factors leading to neuronal death in the neurodegenerative diseases. Ferritin is a hollow iron storage protein composed of 24 subunits of two types, ferritin heavy chain (FTH) and ferritin light chain (FTL), which plays an important role in maintaining iron homeostasis. Recently, the discovery of extracellular ferritin and ferritin in exosomes indicates that ferritin might be not only an iron storage protein within the cell, but might also be an important factor in the regulation of tissue and body iron homeostasis. Read More

View Article and Full-Text PDF
January 2021

Light chain deposition disease involving kidney and liver in a patient with IgD myeloma.

BMC Nephrol 2021 Jan 23;22(1):40. Epub 2021 Jan 23.

Division of Hematology/Oncology, Department of Internal Medicine, Kameda Medical Center, 929 Higashi-chou, Kamogawa-shi, Chiba, 296-8601, Japan.

Background: IgD multiple myeloma (MM) is a rare subtype of MM and light chain deposition disease (LCDD) outside the kidney is also a rare and has scarcely been reported. We report herein the details of the first reported case of LCDD involving the kidney and liver co-occurring with IgD myeloma.

Case Presentation: A 66-year-old female with IgD MM presented with rapidly progressive acute renal failure, ascites and pleural effusion. Read More

View Article and Full-Text PDF
January 2021

The not so innocent bystander: an unusual cause of progressive breathlessness.

Thorax 2021 Jan 21. Epub 2021 Jan 21.

Bristol Interstitial Lung Disease Service, North Bristol NHS Trust, Bristol, UK

This case report discusses a 76-year-old man who presented with symptomatic diffuse alveolar-septal and tracheobronchial amyloidosis with a low-grade monoclonal gammopathy. This patient had a combination of both symptomatic diffuse alveolar-septal interstitial disease and tracheobronchial amyloidosis, features that contradict the widely accepted presentations seen in this disease. First, tracheobronchial amyloidosis has been documented as localised disease without systemic involvement. Read More

View Article and Full-Text PDF
January 2021

[Multiple Cysts Formation in Pulmonary Light Chain Deposition Disease(LCDD)-A Case Report].

Gan To Kagaku Ryoho 2020 Dec;47(13):1997-1999

Dept. of Surgery, Tokyo Metropolitan Otsuka Hospital.

Aim: To review the clinical features of the patient with cystic pulmonary light chain deposition disease(LCDD)and record high-resolution CT and histopathological findings.

Patient: A 60-year-old woman who diagnosed multiple myeloma was admitted. There were diffuse proliferation of plasma cells by the bone marrow puncture that she received. Read More

View Article and Full-Text PDF
December 2020

Skeletal Myopathy as the Initial Manifestation of Light Chain Multiple Myeloma.

Eur J Case Rep Intern Med 2020 20;7(12):002095. Epub 2020 Nov 20.

Pathologic Anatomy and Cytology Laboratory, University Hospital Center Ibn Rochd, Casablanca, Morocco.

Monoclonal gammopathies due to plasma cell dyscrasias can cause various rare neuromuscular disorders. The peripheral nervous system is most commonly affected, while muscle diseases associated with monoclonal gammopathies are rare. Skeletal myopathy, as a manifestation in the context of multiple myeloma, is extremely uncommon and is usually the result of immunoglobulin light chain (AL) amyloidosis deposits in the muscles. Read More

View Article and Full-Text PDF
November 2020