A Amiloidose AL é uma doença das células plasmáticas (um tipo de glóbulo branco) caracterizada pelo acúmulo e depósito de fibrilas amiloides insolúveis, que são proteínas anormais que não se dissolvem. Elas se formam a partir de partes específicas de anticorpos (conhecidas como cadeias leves de imunoglobulina) que se dobram de maneira errada, sendo geralmente produzidas por um tumor dessas células plasmáticas. A doença normalmente se manifesta como amiloidose sistêmica primária (ASP), quando afeta múltiplos órgãos, e com menos frequência como amiloidose localizada primária (ALP), restrita a um único órgão.
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A Amiloidose AL é uma doença das células plasmáticas (um tipo de glóbulo branco) caracterizada pelo acúmulo e depósito de fibrilas amiloides insolúveis, que são proteínas anormais que não se dissolvem. Elas se formam a partir de partes específicas de anticorpos (conhecidas como cadeias leves de imunoglobulina) que se dobram de maneira errada, sendo geralmente produzidas por um tumor dessas células plasmáticas. A doença normalmente se manifesta como amiloidose sistêmica primária (ASP), quando afeta múltiplos órgãos, e com menos frequência como amiloidose localizada primária (ALP), restrita a um único órgão.
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Mostrando amostra de 200 publicações de um total de 2.185
Blood phosphorylated tau elevation as a biomarker in immunoglobulin light chain and transthyretin amyloidosis.
Elevated blood levels of phosphorylated tau (p-tau) are diagnostic of Alzheimer disease and are associated with the deposition of amyloid-β in the cerebral neuropil. Elevated p-tau levels have also been associated with cerebral deposition of Danish amyloid and prion protein amyloid. Here we analyzed p-tau in serum from four different cohorts of people with the most common types of systemic amyloidosis, transthyretin (ATTR) amyloidosis and immunoglobulin light chain (AL) amyloidosis. We found higher levels of serum p-tau181 in the AL and ATTR groups than in controls. Subsequent analyses revealed that these effects were more pronounced in the presence of polyneuropathy (PNP) and in AL compared to ATTR amyloidosis. Individuals with different forms of PNP that were not due to amyloidosis did not exhibit elevated p-tau181 levels. In cases of presymptomatic (genetic) ATTR, p-tau181 levels increased as a function of predicted years from symptom onset. Additional measurement of p-tau217 in one cohort revealed similar increases, and discriminated people with AL and those with ATTR from controls equally as well as p-tau181. These findings suggest that elevated serum p-tau levels are not specific to Alzheimer disease and may also serve as a diagnostic tool of ATTR and AL amyloidosis, with potential utility in distinguishing amyloidosis-related PNP from PNP of other etiologies.
Prognostic Factors and Progression Biomarkers in AL Amyloidosis: Mapping Current Knowledge and Critical Gaps.
The therapeutic landscape for systemic immunoglobulin light chain (AL) amyloidosis has been revolutionized by daratumumab-based regimens, achieving 76% five-year overall survival in the landmark ANDROMEDA trial. However, the current prognostic models were developed using patient populations treated with now-suboptimal therapies, creating a critical gap between risk stratification models and contemporary outcomes. This comprehensive review analyses prognostic factors and progression biomarkers in AL, categorizing them into disease-specific (clone-related and organ-related) and patient-specific factors. Notably, traditional baseline biomarkers including difference between involved and uninvolved free light chains (dFLC) and bone marrow plasma cell burden are losing prognostic significance with effective clone-directed therapies. Emerging approaches show promise, including dynamic markers such as minimal residual disease by free light chain mass spectrometry, cardiac imaging parameters such as global longitudinal strain, and functional measures. There is an urgent need for validation studies and prognostic model refinement to identify high-risk patients who may benefit from interventions beyond anti-plasma cell therapy.
Cryo-EM structures of light chain fibrils from abdominal fat biopsies of multiple myeloma patients.
Multiple myeloma (MM) is a hematological malignancy characterized by the abnormal proliferation of plasma cells in the bone marrow and is closely associated with systemic light chain amyloidosis (AL), a disease triggered by the amyloid aggregation of antibody light chain (LC). High-resolution structures of amyloid LC fibrils from MM patients remain largely unexplored. Here, we extracted LC amyloid fibrils directly from abdominal fat biopsies of two living MM patients. Using cryo-electron microscopy (cryo-EM), we further determined the structures of three distinct LC fibril morphologies, which differ significantly from previously reported fibril structures derived from AL amyloidosis patients. Structural comparisons and computational analyses suggested distinct LC fibrillation mechanisms in MM compared to AL amyloidosis, highlighting a potential role of elevated protein concentration in driving LC aggregation in MM. These findings provide essential molecular insights into LC fibril formation in MM and offer a structural foundation for biological diagnosis of living MM patients based on accessible abdominal fat biopsy samples.
Invasive pressure-volume loop and PET-MR phenotyping in transthyretin cardiac amyloidosis: a multimodal imaging case report.
Cardiac amyloidosis (CA) is an under-recognized cause of heart failure in elderly patients. While diagnosis has traditionally relied on echocardiographic red flags and bone scintigraphy, novel tools may provide enhanced disease characterization. We present the case of a 78-year-old man with progressive symptoms of heart failure who was diagnosed with wild-type transthyretin cardiac amyloidosis (ATTRwt-CM) through conventional and advanced multimodal imaging. Initial clues included a discordance between QRS voltages on electrocardiography and increased left ventricular wall thickness on echocardiography, along with signs of elevated filling pressures. Speckle-tracking echocardiography revealed impaired regional myocardial deformation, global function, and work energetics. Serum and urine immunofixation excluded light chain (AL) amyloidosis. DPD scintigraphy confirmed amyloid deposition with a characteristic distribution. Genetic testing ruled out hereditary variants. PET imaging demonstrated myocardial uptake suggestive of amyloid infiltration and microcalcification. Cardiac MR revealed elevated native T1 and extracellular volume fractions. Invasive pressure-volume loop assessment confirmed biventricular stiffness and impaired contractile reserve, despite clinical compensation at rest. These findings supported early initiation of Tafamidis in a minimally symptomatic patient. This case highlights the role of advanced diagnostics in refining cardiac amyloidosis phenotyping and guiding individualized therapeutic decisions.
Pleural amyloidosis: timely unmasking of apple-green birefringence in pleural biopsy.
8%-25% of cases of pleural effusion remain undiagnosed after routine workup. Some of the causes for pleural effusion are less often considered as a differential diagnosis owing to their rarity. While not all require a pleural biopsy, it remains a valuable tool for the definitive diagnosis of uncommon pleural effusions and guides treatment decisions. This case exemplifies the value of timely intervention in uncovering an unconventional form of pleural amyloidosis. An elderly male presenting with right-sided pleural effusion. The patient underwent timely medical thoracoscopy and was subsequently diagnosed with AL amyloidosis. Following therapy, the patient demonstrated marked clinical improvement, with radiographic resolution of the effusion and a favourable haematologic response.
Publicações recentes
Risk of AL Amyloidosis is Associated with Degree of Free Light Chain Elevation and Duration of Exposure.
Diagnostic Utility of Immunofixation and Free Light Chain Assay in AL Amyloidosis: A Systematic Review and Meta-analysis.
Tailoring heart failure management in cardiac amyloidosis.
Determinants of Gastrointestinal Disease Burden and Survival in Systemic AL Amyloidosis.
Rare dual-clone phenomenon: concurrent λ AL amyloidosis and κ-restricted Bcell lymphoproliferative disorders.
📚 EuropePMC1.519 artigos no totalmostrando 198
The Safety and Efficacy of Commercial BCMA-Directed CAR T-Cell Therapy in Systemic AL Amyloidosis With Concurrent Myeloma.
American journal of hematologyCharacteristics and Clinical Significance of Myocardial Work in Cardiac Light-Chain Amyloidosis: Pressure-Volume Loop Analysis Based on Cardiac Magnetic Resonance.
Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic ResonanceLong-Term Outcomes in Patients with Renal Systemic Light Chain Amyloidosis.
Kidney diseases (Basel, Switzerland)Daratumumab plus bortezomib and dexamethasone (Dara-VD) in newly diagnosed Mayo 2004 stage IIIA and IIIB light-chain amyloidosis: Long-term follow-up results from a prospective phase 2 study.
British journal of haematologyMonoclonal Gammopathies of Thrombotic and Hemorrhagic Significance: Mapping into a classification schema.
Journal of thrombosis and haemostasis : JTHTeclistamab Treatment Followed by Heart Transplantation for Advanced Immunoglobulin Light Chain Amyloid Cardiomyopathy.
JACC. Case reportsA Case of Amyloid Light-Chain Amyloidosis Presenting as Colitis.
CureusZanubrutinib in AL Amyloidosis Associated With Waldenström Macroglobulinemia and Other B-Cell Non-Hodgkin Lymphoma.
American journal of hematologyTalquetamab induces deep responses in heavily pre-treated patients with systemic light-chain amyloidosis.
Annals of hematologyBlood phosphorylated tau elevation as a biomarker in immunoglobulin light chain and transthyretin amyloidosis.
Nature medicineEarly treatment switch in cardiac AL amyloidosis: a case of suboptimal response to first-line Dara-CyBorD regimen followed by switch to teclistamab.
Leukemia & lymphomaLong-term outcomes of autologous stem cell transplantation for AL amyloidosis: a 15-year experience from a Chinese referral center and a comparison of different eras.
Blood cancer journalRight Diaphragmatic Paralysis as Initial Manifestation of AL Amyloidosis.
The American journal of medicineClinical impact of lilac-colored aggregates on may-Grünwald-Giemsa-stained bone marrow smears in patients with amyloid light-chain amyloidosis.
Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of AmyloidosisNon-invasive differentiation of light chain amyloidosis and multiple myeloma based on Raman spectroscopy analysis using one-dimensional convolutional neural networks.
Spectrochimica acta. Part A, Molecular and biomolecular spectroscopyPredicting Early Mortality in Newly Diagnosed AL Amyloidosis: Development and Validation of the PACE Score for Daratumumab-Treated Patients.
American journal of hematologyA Case of AL Amyloidosis-Associated Arthritis Initially Misdiagnosed as Rheumatoid Arthritis.
Modern rheumatology case reportsPrognostic Factors and Progression Biomarkers in AL Amyloidosis: Mapping Current Knowledge and Critical Gaps.
BloodSodium-Glucose Cotransporter 2 Inhibitors in Patients with Heart Failure and Transthyretin or Light Chain Amyloidosis: A Real-World Analysis.
Arquivos brasileiros de cardiologiaComparative evaluation of free light chain assays in AL amyloidosis: Performance of Sebia versus Freelite and N-Latex.
Practical laboratory medicineCurrent Standards and Perspectives in Proteomics for Cardiac Amyloidosis.
Analytical chemistryCase Report: Targeted interleukin-6 blockade by siltuximab for cytokine release syndrome control and infection limitation in thirteen patients treated with bi-specific T-cell engagers.
Frontiers in immunologyNegative [99mTc]Tc -DPD Scintigraphy, Presence of Monoclonal Protein and Biopsy Suggestive of AL Amyloidosis in a Patient With Homozygous p.Ala101Val Transthyretin Gene Variant.
Clinical case reportsAL Amyloidosis Patients Continue to Benefit from HDCT/ASCT Consolidation in the Daratumumab Era.
Journal of clinical medicineAssociation Between Common Variable Immunodeficiency and Pulmonary Amyloidosis: Review.
Journal of clinical medicineThe Evolving Landscape of Anti-Clonal Therapy in Newly Diagnosed Systemic Light-Chain (AL) Amyloidosis: Evidence- and Time-Based Comparison with Multiple Myeloma.
Life (Basel, Switzerland)Concurrence of renal amyloidosis and membranous nephropathy: a case series and literature review.
BMC nephrologyValidity of Amyloidosis in the Danish National Patient Registry.
Clinical epidemiologyA retrospective real-world study assessing diagnostic pattern of light-chain amyloidosis in Japan based on data from the medical data vision claims database.
Therapeutic advances in hematologyUtilization and Prognosis of Cardiac Device Implantation in AL Versus ATTR Amyloidosis.
Pacing and clinical electrophysiology : PACEMonoclonal gammopathies of cutaneous significance: A nomenclature and pathophysiology-based classification.
Journal of the American Academy of DermatologyCryo-EM structures of light chain fibrils from abdominal fat biopsies of multiple myeloma patients.
Nature communicationsInvasive pressure-volume loop and PET-MR phenotyping in transthyretin cardiac amyloidosis: a multimodal imaging case report.
European heart journal. Case reportsCase Report: Contrast adenosine stress echocardiography revealing microvascular dysfunction in cardiac AL amyloidosis.
Frontiers in cardiovascular medicineAn Australian Amyloidosis Network analysis of Australian patients with immunoglobulin light chain amyloidosis treated with bortezomib-based chemotherapy.
Internal medicine journalImmunoglobulin Light Chain Amyloidosis: 2026 Update on Diagnosis, Prognosis, and Treatment.
American journal of hematologyHematologic response and kidney outcomes of monoclonal gammopathy of renal significance excluding AL amyloidosis: a report of six cases.
BMC nephrologyA Phase 1 Study of Daratumumab, Ixazomib, and Dexamethasone in AL Amyloidosis.
American journal of hematologyHepatic-Predominant Immunoglobulin G Lambda Light Chain Amyloidosis Associated With Multiple Myeloma: A Report of an Exceptionally Rare Case.
CureusClinical practice pattern of management of plasma cell dyscrasia for kidney transplant candidates and recipients in the United States.
Journal of onco-nephrologyEligibility criteria for clinical trials in AL amyloidosis result in exclusion of nearly half of real-world patients.
HemaSphereCase Report: Image-enhanced endoscopic characteristics of gastric amyloidosis with narrow-band imaging comparison.
Frontiers in medicineClinical characteristics and prognosis of immunoglobulin light chain amyloidosis patients.
Frontiers in medicineOutcomes of patients with systemic light chain amyloidosis treated with DVD front-line therapy.
Cardio-oncology (London, England)Extracellular Vesicles in Cardiac Amyloidosis: From Pathogenesis to Clinical Applications.
Diagnostics (Basel, Switzerland)Monoclonal gammopathy of renal significance in western China: A large cohort study dominated by amyloidosis with distinct clinical outcomes.
Clinics (Sao Paulo, Brazil)Prognostic Impact of Bone Marrow Plasma Cell Percentage in Patients With Systemic Light-Chain Amyloidosis: A Systematic Review and Meta-Analysis.
European journal of haematologyThe impact of cardiac amyloidosis on patients with multiple myeloma: a systematic review and meta-analysis.
Cardio-oncology (London, England)Bridging the Gap in AL and ATTR Cardiac Amyloidosis: Integrating Histopathology, Biomarkers, and Multimodal Imaging for Subtype-Specific Diagnosis.
Reviews in cardiovascular medicineDiagnostic Journeys and Delays in AL Amyloidosis: Insights From a Cross-Sectional Patient Survey.
Clinical lymphoma, myeloma & leukemiaPleural amyloidosis: timely unmasking of apple-green birefringence in pleural biopsy.
BMJ case reports[Light chain amyloidosis: multidisciplinary management and innovative therapeutic strategies. SIC/ANMCO Consensus document of the Italian Cardiac Amyloidosis Network (RIAC)].
Giornale italiano di cardiologia (2006)Bridging Diagnostic Gaps in AL Cardiac Amyloidosis: The Emerging Role of 68Ga-FAPI PET/CT.
Clinical nuclear medicineMyopathies Associated With Monoclonal Gammopathies of Clinical Significance: A Narrative Review.
CureusRaccoon eyes and macroglossia in AL amyloidosis.
QJM : monthly journal of the Association of PhysiciansImpact of Kidney Histology and Choice of Treatment on Survival in Patients with Multiple Myeloma.
Kidney360Primary (AL) Amyloidosis Following COVID-19 Infection: A Case Report.
Clinical case reportsChallenging features of left ventricular wall thickening in a young patient with multiple myeloma and shock: when magnetic resonance imaging makes the difference-a case report.
European heart journal. Case reportsClinical Features of Systemic Amyloidosis: A Scoping Review.
Blood advancesRight Ventricular-Pulmonary Artery Coupling as a Prognostic Marker in Cardiac Amyloidosis: A Comprehensive Review.
Life (Basel, Switzerland)American Society of Hematology (ASH) 2026 Guidelines on Diagnosis of Light Chain Amyloidosis.
Blood advancesPast, Present, and Future of Dexamethasone in Multiple Myeloma and AL Amyloidosis.
Journal of clinical oncology : official journal of the American Society of Clinical OncologyClarifying the immunoglobulin light chain variable gene usage in Chinese patients with renal AL amyloidosis.
Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of AmyloidosisSystemic light chain cardiac amyloidosis with atrioventricular block.
Frontiers in cardiovascular medicineSurgical Treatment of Primary Localized Cutaneous Nodular Amyloidosis of the Nose: Resection and Reconstruction With a Nasolabial Flap.
CureusFrequency-adjusted daratumumab-based regimen versus bortezomib/dexamethasone in newly diagnosed AL amyloidosis: a matched-cohort study.
Annals of medicineImpact of disease-modifying therapies on imaging parameters in cardiac amyloidosis: A systematic review and meta-analysis.
Current problems in cardiologyNodular pulmonary amyloidosis presenting as a localized manifestation of lymphoproliferative disease: A three-case series.
Respiratory medicine case reportsSmall molecule stabilization of diverse amyloidogenic immunoglobulin light chains revealed by hydrogen-deuterium exchange mass spectrometry.
bioRxiv : the preprint server for biologyDermatological Manifestations of Amyloid Light-Chain (AL) Amyloidosis: A Case Report Highlighting Early Diagnosis and Treatment.
CureusHepatic response criteria in light chain amyloidosis: a multicenter validation study.
HaematologicaEarly Diagnosis of ATTR-CM Using Carpal Tunnel Biopsy Examination: EDUCATE: A United Kingdom Prospective Multicenter Study.
JACC. Heart failureHepatic Response Dynamics in Newly Diagnosed Patients with Light-chain Amyloidosis: A Retrospective Cohort Study.
Advances in therapyPreclinical and preliminary clinical evaluation of [18F]Florbetazine for non-invasive PET detection of cardiac amyloidosis.
European journal of nuclear medicine and molecular imagingMultimodality Imaging in Cardiac Amyloidosis.
Journal of clinical medicineBiopsy-resolved cryo-EM structures of amyloid fibrils provide molecular insights into AL amyloidosis.
Proceedings of the National Academy of Sciences of the United States of AmericaSilent progression: cardiac amyloidosis unmasking IgG lambda myeloma in an elderly patient.
The Egyptian heart journal : (EHJ) : official bulletin of the Egyptian Society of CardiologyPrognostic significance of cardiac biomarkers in patients with AL amyloidosis treated with a daratumumab-based regimen.
Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of AmyloidosisThe role of cardiac imaging for diagnosis of cardiac amyloidosis: a systematic review and meta-analysis of test accuracy.
Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of AmyloidosisSelective Cardiac Tc-99m DPD Retention With Markedly Reduced Extracardiac Uptake in Hereditary Transthyretin Amyloidosis.
Clinical nuclear medicineKinetics of hematologic response in AL amyloidosis: Insights from clinical and cytogenetic subgroup analysis in the daratumumab era.
HemaSphereProving Bone Marrow Plasma Cell Clonality by Flow Cytometry: An Important Tool in the Diagnosis of Immunoglobulin Light-Chain Amyloidosis.
BiomedicinesDetection yield of surrogate tissue biopsies across amyloidosis classes: a large-scale analysis of 4,027 patients.
Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of AmyloidosisRecurrence of light chain crystalline podocytopathy and tubulopathy after kidney transplantation achieving remission with an antimyeloma regimen.
American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant SurgeonsBortezomib combined with autologous stem cell transplantation overcomes cytogenetic abnormalities in AL amyloidosis.
International journal of hematologyALyzer3D.AI: a more generalizable deep learning predictor of light chain amyloidogenicity powered by structural and evolutionary Artificial Intelligence.
Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis2025 Update of Cellular Immunotherapy for Plasma Cell Disorders.
Turkish journal of haematology : official journal of Turkish Society of HaematologyClinical significance of t(11;14) translocation in systemic AL amyloidosis in the era of daratumumab therapy.
Clinical and experimental medicineUntwisted amyloid fibrils from a transgenic mouse model of AL amyloidosis.
Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis[ATTR Amyloidosis for Hand Surgeons: A Hidden, Treatable Systemic Disease].
Handchirurgie, Mikrochirurgie, plastische Chirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft fur Handchirurgie : Organ der Deutschsprachigen Arbeitsgemeinschaft fur Mikrochirurgie der Peripheren Nerven und Gefasse : Organ der V...Coronary microvascular dysfunction as an initial clue to the diagnosis of light-chain amyloidosis: a case report.
European heart journal. Case reportsCryo-EM structure of renal AL amyloid fibrils from a patient with λ1 light chain amyloidosis.
Nature communicationsAvoiding serious treatment-emergent adverse events in Waldenström macroglobulinemia patients.
Expert review of hematologyCryo-EM of Cardiac AL-224L Amyloid Reveals Shared Structural Motifs and Mutation-induced Differences in λ6 Light Chain Fibrils.
Journal of molecular biologyLinvoseltamab in Patients With Relapsed/Refractory Multiple Myeloma in the LINKER-MM1 Study: Longer Follow-Up and Subgroup Analyses.
Clinical lymphoma, myeloma & leukemia[Abdominal pain: It still can be a zebra].
PraxisA Case of AL Amyloidosis With Hepatomegaly as the Main Clinical Manifestation.
Clinical case reportsScintigraphy-Positive Light-Chain Cardiac Amyloidosis.
Journal of nuclear medicine technologyUp-to-date review on heart transplantation and other advanced heart failure therapies in cardiac amyloidosis patients.
Future cardiologyNew Validated Staging System for Light Chain (AL) Amyloidosis With Stage IIIC Defining Ultra-Poor Risk: AL International Staging System.
Journal of clinical oncology : official journal of the American Society of Clinical OncologyMonoclonal-related neuropathies: diagnosis, prognosis, and outcomes.
Hematology. American Society of Hematology. Education ProgramEndless possibilities and how to exploit them? What is the optimal treatment sequence?
Hematology. American Society of Hematology. Education ProgramGeometry over thickness: Refining echocardiographic recognition of amyloid cardiomyopathy.
Progress in cardiovascular diseasesFrontline Dara-CyBorD for AL amyloidosis: high response rates and cytogenetic insights from a real-world cohort.
Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of AmyloidosisPulmonary Amyloidosis as a Differential Diagnosis in Interstitial Lung Diseases.
Open respiratory archivesReal-World Data of Light Chain (AL) Amyloidosis: Prognostic Indices and Treatment Patterns.
BiomedicinesA Triad of Telltale Clues: Macroglossia Raccoon Eye and Nerve Compression Unveil Amyloid Light Chain Amyloidosis.
The Journal of the Association of Physicians of IndiaRethinking response assessment in AL amyloidosis: the role of kidney function and absolute light chain levels.
Leukemia & lymphomaPolymorphic IGLV6-57 AL amyloid fibrils and features of a shared folding pathway.
bioRxiv : the preprint server for biologyThe impact of growth factor utilization on infection risk in patients receiving bispecific monoclonal antibodies.
Journal of oncology pharmacy practice : official publication of the International Society of Oncology Pharmacy PractitionersSingle-slide detection and typing of AL renal amyloidosis: combining mass spectrometry imaging and digital pathology.
Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of AmyloidosisOptimized methods for efficient application of immunogold electron microscopy to amyloid fibrils typing.
Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of AmyloidosisIsatuximab: surface strike, deep response in AL amyloidosis.
BloodIsatuximab for relapsed AL amyloidosis.
BloodAmyloid Deposits in a Bone Marrow Biopsy Alongside a Presumed Causative Clone.
EJHaemDevelopment of an artificial intelligence-based early diagnostic system for light-chain amyloidosis.
Annals of hematologyThe Real-World Safety and Efficacy of Bispecific T-Cell Engager Therapy in Systemic AL Amyloidosis.
American journal of hematologyThe Association of Kidney Function on Survival in Patients with AL Amyloidosis after Autologous Stem Cell Transplantation: A Multicenter Study.
Kidney360Cardiac Amyloidosis in the Real World: Clinical Presentations, Disease Overlap, and Therapeutic Imperatives.
Reviews in cardiovascular medicineEfficacy of venetoclax in the treatment of relapsed/refractory light-chain amyloidosis.
Annals of hematologyEquivocal vs Positive Technetium-99m-Pyrophosphate Scintigraphy for Transthyretin Amyloid Cardiomyopathy: Comparing Outcomes, Demographics, and Imaging.
CJC openTwin Diseases in Different Organs: A Cautionary Tale of Anchoring on a Positive Scan.
CirculationImpact of early minimal residual disease assessment on the outcomes of patients with systemic light chain amyloidosis.
Annals of medicineNovel noninvasive parameters for diagnosis of liver involvement in patients with systemic AL amyloidosis.
International journal of hematologyCold pressor test and paradoxical blood pressure reduction in light chain amyloidosis.
Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of AmyloidosisDecoding the human tongue of light chain amyloidosis in three dimensions (3D): three-dimensional pathological visualization drives early and precise screening of suspected cases.
BMC oral healthWild-Type Transthyretin Amyloid Cardiomyopathy With Bone Marrow Involvement: Clinical Implications of Less Common Sites of Amyloid Deposition.
CureusCutaneous light chain amyloidosis and erythema elevatum diutinum as the presenting features of IgA monoclonal gammopathy: a rare case report and comprehensive literature review.
Annals of hematologyAmyloid and Light Chain Deposition Disorders.
Clinics in chest medicine[A rare cause of hoarseness-a case study and compact overview of laryngeal amyloidosis].
HNOBCMA: BeCoMing a new hope for AL amyloidosis.
BloodFirst case report of talquetamab use in AL amyloidosis.
Leukemia & lymphomaSerum and Tissue Light-Chains as Disease Biomarkers in AL Amyloidosis.
International journal of molecular sciencesAL amyloidosis: a real-world experience with CAR T-cell therapy.
Blood advancesProfound dFLC decrease after one cycle predicts superior outcome in patients with AL amyloidosis.
Leukemia & lymphomaPrimary Amyloidosis Unmasked by Gastric Biopsy: A Case Report.
Clinical medicine insights. Case reportsAssessment of Treatment Response: Expanding the Applications of 18F-Florbetapir PET Imaging in Light-Chain Cardiac Amyloidosis Beyond Diagnosis and Outcome Prediction.
JACC. Cardiovascular imagingPerformance of Large Language Models in Diagnosing Rare Hematologic Diseases and the Impact of Their Diagnostic Outputs on Physicians: Combined Retrospective and Prospective Study.
Journal of medical Internet researchUrinary Stricture due to Localized Urethral AL Amyloidosis: A Case Report and Literature Review.
Case reports in oncologyDimer Dissociation and Aggregation Hot-spot Exposure Synergistically Accelerate Light Chain Variable Domain Aggregation Associated With AL Amyloidosis.
Journal of molecular biologyMonoclonal Gammopathy of Clinical Significance-Associated Glycogen Storage Myopathy: A Novel Acquired Muscle Disease.
CureusMultisystem Light Chain Amyloidosis: Diagnostic and Therapeutic Challenges.
CureusCardiac AL amyloidosis in a veteran endurance athlete with pre-existing apical hypertrophic cardiomyopathy: a case report.
European heart journal. Case reportsDiagnostic Dilemmas in Cardiac Transthyretin Amyloidosis With Coexistent Low-Grade B-Cell Lymphoma: A Case Report.
CureusEvaluation of Cytokine Levels in Cardiac Transthyretin and Immunoglobulin Light Chain Amyloidosis and Their Correlation with Myocardial Inflammatory Cells and MACE.
BiomedicinesDiet-related poor nutritional status as a major challenge in the treatment of patients with amyloidosis: A systematic review.
Nutrition (Burbank, Los Angeles County, Calif.)Bleeding Patterns and Clinical Outcomes in Patients With Systemic AL Amyloidosis-Related Acquired Factor X Deficiency.
American journal of hematologyMyocardial deformation imaging to monitor treatment response in AL amyloidosis: is it worth the strain?
European heart journal openImprovement in global longitudinal strain following plasma cell-directed therapy is associated with long-term survival among patients with AL amyloidosis.
European heart journal openMolecular Expression Differences in Specific Blood Mononuclear Cell-Types Identify Patients With AL Amyloidosis.
Journal of cellular and molecular medicineUnmasking amyloid light-chain amyloidosis through biochemical lens: diagnostic utility of urine immunofixation in serum-negative cases.
Diagnosis (Berlin, Germany)Dangerous clones with complex ties to AL amyloidosis.
BloodSimultaneous onset of systemic sclerosis and light chain amyloidosis: the first report of a case report and review of the literature.
Rheumatology internationalEchocardiographic AMYLI score in systemic light-chain amyloidosis: Clinical relevance and risk stratification.
International journal of cardiologyDiagnostic Performance of the New Free Light Chain Ratio in Systemic Amyloidosis.
JACC. CardioOncologyChanges in Myocardial Light Chain Amyloid Burden After Plasma Cell Therapy.
JACC. Cardiovascular imagingIsatuximab for relapsed and/or refractory AL amyloidosis: results of a prospective phase 2 trial (SWOG S1702).
BloodClinicopathologic Features, Pathogenesis, and Treatment of Monoclonal Gammopathy-Associated Myopathies.
NeurologyA tale of two tracers - amyloid imaging with investigational radiotracers iodine (124I) evuzamitide and 99mTc-p5+14 (AT-05).
Journal of nuclear cardiology : official publication of the American Society of Nuclear CardiologyConcurrent TTR and AL Amyloidosis: Diagnostic Challenges and Clinical Implications.
JACC. Case reportsCardiac light chain amyloidosis secondary to Waldenström's macroglobulinemia: A case report and review.
Experimental and therapeutic medicineComparable peri-transplant mortality and incidence of febrile neutropenia after high-dose melphalan and autologous stem cell transplantation in patients with plasma cell dyscrasias treated as outpatient versus inpatient.
British journal of haematologyCase report of isolated aortic valve AL-amyloidosis following aortic valve replacement.
European heart journal. Case reportsPredicting prognosis of light-chain cardiac amyloidosis by magnetic resonance imaging and deep learning.
European heart journal. Cardiovascular ImagingThe Conformational Landscape of AlphaFold2-Predicted Amyloidogenic Light Chains and Their Correlation With VL Domain Mutations and Aggregation Propensity.
Journal of molecular recognition : JMRIn vitro and in vivo disease models of cardiac amyloidosis: progress, pitfalls, and potential.
Cardiovascular research[Clinical characteristics analysis of patients with IgD type systemic light chain amyloidosis].
Zhonghua yi xue za zhiRapidly progressive fatal hepatic failure due to IgM-related AL amyloidosis in Waldenström's macroglobulinemia: an autopsy case report.
Journal of clinical and experimental hematopathology : JCEHBCMA CAR-T: From Multiple Myeloma to Light-Chain Amyloidosis.
Current oncology (Toronto, Ont.)Clone-specific residue changes at multiple positions are associated with amyloid formation by antibody light chains.
Frontiers in immunologyAdvances in imaging-based diagnosis, prognosis, and response assessment in cardiac amyloidosis: a comprehensive multimodality review.
Annals of nuclear medicineWild-type transthyretin cardiac amyloidosis: a case of multisystemic involvement and review of literature.
Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologieA rare presentation of extramedullary multiple myeloma as a large gluteal soft tissue mass: A case report.
International journal of surgery case reportsBeyond the Usual Suspects: Clinical and Pathological Insights From a Rare Case of Light Chain (AL) Amyloidosis in a Filipino Patient.
CureusUnexplained cardiac hypertrophy as a clue to plasma cell tumour: a case study.
American journal of translational researchLocalized Nodular Amyloidosis of the Buccal Mucosa Associated with Sjögren's Syndrome: A Case Report and Literature Review.
Yonago acta medicaConcurrent Immunoglobulin Light Chain and Transthyretin Cardiac Amyloidosis Patient Treated With Daratumumab and Tafamidis: A Case Report.
EJHaemTherapeutic role of venous leg compression in worsening heart failure with predominant extravascular congestion: a case report.
European heart journal. Case reportsUltrasonographic Features of Amyloid Arthropathy in Light Chain Amyloidosis: A Case Report.
Current rheumatology reviewsPrognostic value of right ventricular longitudinal strain assessed by multimodal imaging in amyloidosis: systematic review and meta-analysis.
European heart journal. Cardiovascular ImagingImpact of autonomic dysfunction in light chain amyloidosis patient with nephrotic syndrome and cardiac involvement.
Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of AmyloidosisCharting the future of AL amyloidosis: Diagnostic advancements, prognostic insights, and therapeutic challenges.
European journal of internal medicineSpectrum of renal diseases caused by monoclonal immunoglobulin: experience from an Australian tertiary referral centre over a 10-year period.
PathologyWild-type Cardiac Amyloidosis due to Transthyretin Protein - A Review.
Journal of community hospital internal medicine perspectivesManagement recommendations for kidney transplantation in patients with plasma cell dyscrasia.
Kidney internationalEscherichia coli Cellulitis in a Patient With Waldenström Macroglobulinaemia: A Case Report.
CureusBispecific T-cell engagers for relapsed/refractory multiple myeloma after solid organ transplantation: A case series.
Journal of investigative medicine : the official publication of the American Federation for Clinical ResearchHow to perform and interpret cardiac amyloidosis radionuclide imaging (CARI).
Journal of nuclear cardiology : official publication of the American Society of Nuclear CardiologySafety and efficacy of elranatamab in patients with relapsed and/or refractory immunoglobulin light-chain amyloidosis.
BloodModeling immunoglobulin light chain amyloidosis in Caenorhabditis elegans.
Disease models & mechanismsUsefulness of STREI: A new index of right heart function in patients with immunoglobulin light chain cardiac amyloidosis.
International journal of cardiologyRapid identification of daratumumab interference with M-protein detection using MALDI-TOF mass spectrometry: a case study involving renal light chain amyloidosis.
Practical laboratory medicineCurrent risk stratification and staging of multiple myeloma and related clonal plasma cell disorders.
LeukemiaAccurate MS-Based Diagnostic Amyloid Typing Using Endogenously Normalized Protein Intensities in Formalin-Fixed Paraffin-Embedded Tissue.
Molecular & cellular proteomics : MCPLong-term outcomes of light chain amyloidosis patients receiving heart transplant: A single-center experience.
JHLT openLong-term follow-up of patients with AL amyloidosis treated on a phase 1 trial of CAEL-101.
Blood advancesAtypical Presentation of Systemic Amyloid Light Chain (AL) Amyloidosis.
CureusRecent developments in systemic light-chain amyloidosis prognosis and treatment.
Future cardiologyAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Blood phosphorylated tau elevation as a biomarker in immunoglobulin light chain and transthyretin amyloidosis.
- Prognostic Factors and Progression Biomarkers in AL Amyloidosis: Mapping Current Knowledge and Critical Gaps.
- Cryo-EM structures of light chain fibrils from abdominal fat biopsies of multiple myeloma patients.
- Invasive pressure-volume loop and PET-MR phenotyping in transthyretin cardiac amyloidosis: a multimodal imaging case report.
- Pleural amyloidosis: timely unmasking of apple-green birefringence in pleural biopsy.
- Risk of AL Amyloidosis is Associated with Degree of Free Light Chain Elevation and Duration of Exposure.
- Diagnostic Utility of Immunofixation and Free Light Chain Assay in AL Amyloidosis: A Systematic Review and Meta-analysis.
- Tailoring heart failure management in cardiac amyloidosis.
- Determinants of Gastrointestinal Disease Burden and Survival in Systemic AL Amyloidosis.
- Rare dual-clone phenomenon: concurrent λ AL amyloidosis and κ-restricted Bcell lymphoproliferative disorders.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:85443(Orphanet)
- MONDO:0019438(MONDO)
- GARD:5797(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q4652470(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
