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Current Cardiology Reports Jul 2022This review will explore the role of cardiac imaging in guiding treatment in the two most commonly encountered subtypes of cardiac amyloidosis (immunoglobulin... (Review)
Review
PURPOSE OF REVIEW
This review will explore the role of cardiac imaging in guiding treatment in the two most commonly encountered subtypes of cardiac amyloidosis (immunoglobulin light-chain amyloidosis [AL] and transthyretin amyloidosis [ATTR]).
RECENT FINDINGS
Advances in multi-parametric cardiac imaging involving a combination of bone scintigraphy, echocardiography and cardiac magnetic resonance imaging have resulted in earlier diagnosis and initiation of treatment, while the evolution of techniques such as longitudinal strain and extracellular volume quantification allow clinicians to track individuals' response to treatment. Imaging developments have led to a deeper understanding of the disease process and treatment mechanisms, which in combination result in improved patient outcomes. The rapidly expanding treatment regimens for cardiac amyloidosis have led to an even greater reliance on cardiac imaging to help establish an accurate diagnosis, monitor treatment response and aid the adjustment of treatment strategies accordingly.
Topics: Amyloid Neuropathies, Familial; Cardiac Imaging Techniques; Cardiomyopathies; Echocardiography; Humans; Immunoglobulin Light-chain Amyloidosis
PubMed: 35524881
DOI: 10.1007/s11886-022-01703-7 -
Giornale Italiano Di Nefrologia :... Oct 2023Immunoglobulin Light Chain Amyloidosis (AL) is a progressive disease which leads to organ dysfunction and death. Tremendous progress has been made in staging, response,... (Review)
Review
Immunoglobulin Light Chain Amyloidosis (AL) is a progressive disease which leads to organ dysfunction and death. Tremendous progress has been made in staging, response, and treatment. The key to better survival though is early diagnosis which can be difficult since the symptoms are often nonspecific and can be seen in more common conditions. Once the diagnosis is confirmed, staging systems are available to provide prognosis on overall and renal survival. There are a number of treatments now available that are effective and well-tolerated. Response criteria have also been developed for hematologic and renal response in order to maximize response and minimize adverse effects. Newer therapies are being developed in particular anti-fibril therapies that are in clinical trials. For those patients who had a very good partial response or better, kidney transplantation may be an option if the kidney failure is not reversed.
Topics: Humans; Immunoglobulin Light Chains; Amyloidosis; Immunoglobulin Light-chain Amyloidosis; Kidney; Kidney Transplantation
PubMed: 38007836
DOI: No ID Found -
Current Treatment Options in Oncology Jul 2022Light-chain amyloidosis is a rare disorder where a small clone of plasma cells is producing excess toxic light chains that deposit in various organs and cause... (Review)
Review
Light-chain amyloidosis is a rare disorder where a small clone of plasma cells is producing excess toxic light chains that deposit in various organs and cause dysfunction. Cardiac involvement is a major determinant of survival and rapid reduction of light chain is critical for recovery of organ function and overall survival. Immunotherapy targeting the clonal plasma cells and amyloid fibrils has emerged as a promising candidate. Daratumumab, both alone and in combinations with other anti-myeloma agents, is able to achieve deep hematologic responses and has greatly improved outcomes. Isatuximab, elotuzumab, and CAEL101 have also shown promising results and further studies are ongoing in the frontline as well as the relapsed/refractory setting. The frailty of AL patients and the relapsing/remitting nature of the disease present unique challenges, and the low toxicity of monoclonal antibodies makes them well-suited for these patients. Other immunotherapy agents including chimeric antigen receptor T cells, bispecific antibodies, and antibody-drug conjugates have altered the landscape in treatment of multiple myeloma, and are in the early phase of evaluation in patients with AL amyloidosis with results eagerly awaited.
Topics: Humans; Immunoglobulin Light-chain Amyloidosis; Immunologic Factors; Immunotherapy; Multiple Myeloma; Neoplasm Recurrence, Local
PubMed: 35635625
DOI: 10.1007/s11864-021-00922-4 -
Brain and Nerve = Shinkei Kenkyu No... May 2024AL amyloidosis, derived from amyloidogenic immunoglobulin light chains, is a common type of systemic amyloidosis. Peripheral neuropathy has been identified in 10%-40% of... (Review)
Review
AL amyloidosis, derived from amyloidogenic immunoglobulin light chains, is a common type of systemic amyloidosis. Peripheral neuropathy has been identified in 10%-40% of patients with systemic AL amyloidosis. Definitive diagnosis requires tissue biopsies, including skin, fat, and gastrointestinal samples, as well as amyloid typing. Disease-modifying therapies have been shown to improve patient survival and prevent progressive organ dysfunction.
Topics: Humans; Amyloidosis; Immunoglobulin Light-chain Amyloidosis; Immunoglobulin Light Chains
PubMed: 38741500
DOI: 10.11477/mf.1416202647 -
Methodist DeBakey Cardiovascular Journal 2022Amyloidosis is a disorder of protein misfolding and metabolism in which insoluble fibrils are deposited in various tissues, causing organ dysfunction and eventually... (Review)
Review
Amyloidosis is a disorder of protein misfolding and metabolism in which insoluble fibrils are deposited in various tissues, causing organ dysfunction and eventually death. Out of the 60-plus heterogeneous amyloidogenic proteins that have been identified, approximately 30 are associated with human disease. The unifying feature of these proteins is their tendency to form beta-pleated sheets aligned in an antiparallel fashion. These sheets then form rigid, nonbranching fibrils that resist proteolysis, causing mechanical disruption and local oxidative stress in affected organs such as the heart, liver, kidneys, nervous system, and gastrointestinal tract. Here we review the epidemiology of light chain amyloidosis, the staging, and the concomitant prognostication that is critical in determining the appropriate treatment.
Topics: Amyloidosis; Humans; Immunoglobulin Light-chain Amyloidosis
PubMed: 35414848
DOI: 10.14797/mdcvj.1070 -
Hematology/oncology Clinics of North... Dec 2020The organs affected most commonly by AL amyloidosis are the kidneys and heart, however, liver and gastrointestinal (GI) tract are also commonly affected. Symptoms of GI... (Review)
Review
The organs affected most commonly by AL amyloidosis are the kidneys and heart, however, liver and gastrointestinal (GI) tract are also commonly affected. Symptoms of GI amyloidosis often mimic those of other GI disorders; having a keen awareness of the need to evaluate for amyloidosis is critical in avoiding delay in diagnosis and intervention. GI and liver involvement is associated with significant complications, and challenges in symptomatic management. As with all AL-related organ disease, early systemic treatment can prevent progression of tissue damage and improve outcomes.
Topics: Animals; Diagnostic Imaging; Disease Management; Disease Susceptibility; Gastrointestinal Tract; Humans; Immunoglobulin Light-chain Amyloidosis; Incidence; Liver; Organ Specificity; Prognosis; Symptom Assessment
PubMed: 33518015
DOI: 10.1016/j.hoc.2020.11.001 -
International Journal of Molecular... Oct 2021Systemic AL amyloidosis is a rare complex hematological disorder caused by clonal plasma cells which produce amyloidogenic immunoglobulins. Outcome and prognosis is the... (Review)
Review
Systemic AL amyloidosis is a rare complex hematological disorder caused by clonal plasma cells which produce amyloidogenic immunoglobulins. Outcome and prognosis is the combinatory result of the extent and pattern of organ involvement secondary to amyloid fibril deposition and the biology and burden of the underlying plasma cell clone. Prognosis, as assessed by overall survival, and early outcomes is determined by degree of cardiac dysfunction and current staging systems are based on biomarkers that reflect the degree of cardiac damage. The risk of progression to end-stage renal disease requiring dialysis is assessed by renal staging systems. Longer-term survival and response to treatment is affected by markers of the underlying plasma cell clone; the genetic background of the clonal disease as evaluated by interphase fluorescence in situ hybridization in particular has predictive value and may guide treatment selection. Free light chain assessment forms the basis of hematological response criteria and minimal residual disease as assessed by sensitive methods is gradually being incorporated into clinical practice. However, sensitive biomarkers that could aid in the early diagnosis and that could reflect all aspects of organ damage and disease biology are needed and efforts to identify them are continuous.
Topics: Amyloid; Biomarkers; Disease Progression; Early Diagnosis; Humans; Immunoglobulin Light-chain Amyloidosis; Peptide Fragments; Prognosis; Survival Analysis
PubMed: 34681575
DOI: 10.3390/ijms222010916 -
The American Journal of Cardiology Dec 2021Cardiac amyloidosis is an important clinical entity associated with significant morbidity and mortality. Although the signs and symptoms can be apparent early in the...
Cardiac amyloidosis is an important clinical entity associated with significant morbidity and mortality. Although the signs and symptoms can be apparent early in the disease course, diagnoses are often made late because of inadequate recognition. A diagnosis of cardiac amyloidosis requires careful scrutiny of a patient's symptoms, an electrocardiogram, and imaging studies, including echocardiography and magnetic resonance imaging. Further evaluation is required through the measurement of serum and urine light chains and the use of bone scintigraphy imaging to differentiate transthyretin amyloidosis from light-chain cardiac amyloidosis. The available treatments have expanded tremendously in recent years and have improved outcomes in the population with this disorder. Thus, it has become increasingly important to diagnose cardiac amyloidosis and provide timely therapies. This article will clarify the various misconceptions about cardiac amyloidosis and provide a framework for primary care providers to better identify this disease in their practice.
Topics: Amyloid Neuropathies, Familial; Amyloidosis; Aniline Compounds; Assisted Circulation; Atrial Fibrillation; Cardiac Imaging Techniques; Cardiomyopathies; Diagnosis, Differential; Echocardiography; Electrocardiography; Ethylene Glycols; Humans; Immunoglobulin Light-chain Amyloidosis; Magnetic Resonance Imaging; Positron-Emission Tomography; Radiopharmaceuticals; Stilbenes
PubMed: 34610875
DOI: 10.1016/j.amjcard.2021.08.045 -
Hematology/oncology Clinics of North... Dec 2020Early diagnosis of AL amyloidosis and appreciation of the nutritional and coagulation abnormalities associated with liver and gastrointestinal involvement are critically... (Review)
Review
Early diagnosis of AL amyloidosis and appreciation of the nutritional and coagulation abnormalities associated with liver and gastrointestinal involvement are critically important in the treatment and management. In cases of severe malabsorption total parenteral nutrition can be extremely helpful as a bridge to organ improvement. Rarely the use of antifibrinolytic agents such as oral aminocaproic acid with transfusion support may control severe bleeding in patients with coagulation abnormalities. It is important to keep in mind that organ improvement should follow in lag phase after the reduction in the pathologic free light chain with treatment. Closely following light chain levels may permit brief holidays from treatment and enable periods of recovery before resuming therapy in patients with prompt early and deep hematologic responses.
Topics: Adult; Female; Gastrointestinal Diseases; Humans; Immunoglobulin Light Chains; Immunoglobulin Light-chain Amyloidosis; Liver Diseases; Male; Middle Aged
PubMed: 33099425
DOI: 10.1016/j.hoc.2020.09.001 -
Medicina Clinica Aug 2016
Review
Topics: Anti-Inflammatory Agents; Antineoplastic Agents, Alkylating; Antineoplastic Agents, Immunological; Antineoplastic Combined Chemotherapy Protocols; Combined Modality Therapy; Hematopoietic Stem Cell Transplantation; Humans; Immunoglobulin Light-chain Amyloidosis; Melphalan; Organ Transplantation; Prognosis; Treatment Outcome
PubMed: 27085744
DOI: 10.1016/j.medcli.2016.02.018