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Hematology/oncology Clinics of North... Dec 2020Kidney involvement in immunoglobulin-related amyloidosis (AIg) is common. Although patients with renal-limited AIg tend not to have the high mortality that patients with... (Review)
Review
Kidney involvement in immunoglobulin-related amyloidosis (AIg) is common. Although patients with renal-limited AIg tend not to have the high mortality that patients with cardiac amyloidosis have, they do experience significant morbidity and impact on quality of life. The complexity of the pathogenesis remains incompletely understood. Models have been established to prognosticate and assess for the response to therapy. Patients with advanced renal impairment from immunoglobulin light chain amyloidosis still have poor renal prognosis, and better therapy is needed in order to preserve kidney function. Patients who develop end-stage renal disease can undergo renal replacement therapy with kidney transplantation.
Topics: Antibodies, Monoclonal; Humans; Immunoglobulin Light Chains; Immunoglobulin Light-chain Amyloidosis; Kidney; Kidney Failure, Chronic; Kidney Transplantation; Renal Replacement Therapy
PubMed: 33099424
DOI: 10.1016/j.hoc.2020.08.002 -
Blood Cancer Journal May 2021Immunoglobulin light chain amyloidosis (AL) commonly presents with nephrotic range proteinuria, heart failure with preserved ejection fraction, nondiabetic peripheral... (Review)
Review
Immunoglobulin light chain amyloidosis (AL) commonly presents with nephrotic range proteinuria, heart failure with preserved ejection fraction, nondiabetic peripheral neuropathy, unexplained hepatomegaly or diarrhea, and should be considered in patients presenting with these symptoms. More importantly, patients being monitored for smoldering multiple myeloma and a monoclonal gammopathy of undetermined significance (MGUS) are at risk for developing AL amyloidosis. MGUS and myeloma patients that have atypical features, including unexplained weight loss; lower extremity edema, early satiety, and dyspnea on exertion should be considered at risk for light chain amyloidosis. Overlooking the diagnosis of light chain amyloidosis leading to therapy delay is common, and it represents an error of diagnostic consideration. Herein we provide a review of established and investigational treatments for patients with AL amyloidosis and provide algorithms for workup and management of these patients.
Topics: Amyloid; Animals; Antibodies, Monoclonal; Antineoplastic Agents; Biopsy; Disease Management; Humans; Immunoglobulin Light-chain Amyloidosis; Organ Transplantation
PubMed: 33993188
DOI: 10.1038/s41408-021-00483-7 -
Clinical Nuclear Medicine Mar 2020Amyloidosis is a rare hereditary or acquired protein deposition disorder with different etiologies, characterized by pathological protein deposition essentially in...
Amyloidosis is a rare hereditary or acquired protein deposition disorder with different etiologies, characterized by pathological protein deposition essentially in nearly any organs or tissues. There are 2 major forms: primary and secondary amyloidosis. Moreover, it is possible to have systemic or localized disease. The localized form of amyloidosis affecting the small intestine is rare, and it is characterized by the formation of precursor proteins at the site of the lesion. We report a case of localized small bowel amyloidosis studied by F-choline and F-FDG-iodinated PET/CT performed for staging an aggressive prostatic cancer.
Topics: Aged; Choline; Fluorodeoxyglucose F18; Humans; Immunoglobulin Light-chain Amyloidosis; Intestine, Small; Male; Positron Emission Tomography Computed Tomography; Radiopharmaceuticals
PubMed: 31977458
DOI: 10.1097/RLU.0000000000002889 -
Journal of the National Comprehensive... Jan 2023Systemic light chain (AL) amyloidosis is caused by a B-cell (most commonly plasma cell) clone that produces a toxic light chain that forms amyloid fibrils in tissues and... (Review)
Review
Systemic light chain (AL) amyloidosis is caused by a B-cell (most commonly plasma cell) clone that produces a toxic light chain that forms amyloid fibrils in tissues and causes severe, progressive organ dysfunction. The clinical presentation is protean, and patients are usually extremely frail, thus requiring careful adaptation of the treatment approach. However, the severity of organ involvement can be accurately assessed with biomarkers that allow a sharp prognostic stratification and precise tailoring of the treatment strategy. Moreover, the availability of biomarker-based response criteria also allows adjustment of the treatment approach over time. The recent completion of 3 large randomized clinical trials has offered new evidence for designing appropriate treatments. All this information has recently been integrated in the joint guidelines of the International Society of Amyloidosis and the European Hematology Association for the treatment of AL amyloidosis. Other clinical trials are underway testing new agents directed against the amyloid clone and the amyloid deposits. Our understanding of the peculiarities of the amyloid clone, as well as our ability to detect residual clonal disease and improve organ dysfunction, are also being refined and will result in more precise personalization of the treatment approach.
Topics: Humans; Multiple Organ Failure; Amyloidosis; Immunoglobulin Light-chain Amyloidosis; Amyloid; Prognosis; Biomarkers
PubMed: 36634608
DOI: 10.6004/jnccn.2022.7092 -
European Journal of Internal Medicine Dec 2018Systemic immunoglobulin light chain (AL) amyloidosis is an aging-associated protein misfolding and deposition disease. This condition is caused by a small and otherwise... (Review)
Review
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. Therefore, its diagnosis requires a high degree of clinical suspicion and reliable diagnostic tools for accurate amyloid typing, available at specialized referral centers. In AL amyloidosis, frailty is dictated by the type and severity of organ involvement, with heart involvement being the main determinant of morbidity and mortality. Still, given a similar disease stage, elderly patients with AL amyloidosis are often an even frailer group, due to significant comorbidities, associated disability and polypharmacotherapy, socioeconomic restrictions, and limited access to clinical trials. Recent improvements in the use of biomarkers for early diagnosis, risk stratification and response monitoring, the flourishing of novel, effective anti-plasma cell therapies developed against multiple myeloma and adapted to treat AL amyloidosis, and possibly the introduction of anti-amyloid therapies are rapidly changing the clinical management of this disease and are reflected by improved outcomes. Of note, hematologic and organ responses in elderly patients with AL amyloidosis do translate in better outcome, advocating the importance of treating these patients and striving for a rapid response to therapy also in this challenging clinical setting.
Topics: Aged; Biomarkers; Diagnosis, Differential; Disease Management; Frail Elderly; Geriatric Assessment; Humans; Immunoglobulin Light-chain Amyloidosis
PubMed: 29801808
DOI: 10.1016/j.ejim.2018.05.004 -
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 -
Lancet (London, England) Jul 1953
Topics: Amyloidosis; Disease; Heart Failure; Humans; Immunoglobulin Light-chain Amyloidosis; Neuritis; Peripheral Nerves
PubMed: 13062603
DOI: 10.1016/s0140-6736(53)91089-7 -
Geriatrics Apr 1964
Topics: Amyloidosis; Clinical Laboratory Techniques; Coloring Agents; Dermatology; Gastroenterology; Geriatrics; Heart Diseases; Humans; Immunoglobulin Light-chain Amyloidosis; Liver Diseases; Liver Function Tests; Multiple Myeloma; Neoplasms, Plasma Cell; Nephrotic Syndrome; Neurologic Manifestations; Respiratory Tract Diseases; Staining and Labeling
PubMed: 14141809
DOI: No ID Found -
The Lancet. Haematology Nov 2023The primary goal of the initial treatment in systemic light chain amyloidosis is to obtain a rapid and profound haematological response as safely as possible, coupled... (Review)
Review
The primary goal of the initial treatment in systemic light chain amyloidosis is to obtain a rapid and profound haematological response as safely as possible, coupled with supportive care by a multidisciplinary team. The treatment landscape has evolved with the introduction of highly effective therapies targeting the plasma cell clones, which can attain high rates of haematological complete response with minimal treatment-related morbidity and mortality. Consequently, the role of high-dose melphalan followed by autologous haematopoietic cell transplantation (HDM-AHCT) is being analysed, particularly considering the absence of randomised controlled trial data supporting its superiority over standard-dose therapies in systemic light chain amyloidosis treatment. In this Viewpoint, we will explore the role of HDM-AHCT in the management of patients with systemic light chain amyloidosis who are eligible for transplantation, and the unresolved questions surrounding HDM-AHCT use as both front-line and salvage therapy.
Topics: Humans; Antineoplastic Agents; Immunoglobulin Light-chain Amyloidosis; Amyloidosis; Hematopoietic Stem Cell Transplantation; Antibodies, Monoclonal; Transplantation, Autologous; Treatment Outcome
PubMed: 37802087
DOI: 10.1016/S2352-3026(23)00175-8 -
The Journal of Urology Oct 1964
Topics: Adolescent; Amyloidosis; Humans; Immunoglobulin Light-chain Amyloidosis; Pathology; Ureter
PubMed: 14214467
DOI: 10.1016/S0022-5347(17)63946-6