Gènes de chaine légère d'immunoglobuline : Questions médicales fréquentes
Nom anglais: Genes, Immunoglobulin Light Chain
Descriptor UI:D050439
Tree Number:G05.360.340.024.340.335.310
Questions fréquentes et termes MeSH associés
Diagnostic
5
#1
Comment diagnostiquer une anomalie des chaînes légères ?
Des tests sanguins et des analyses génétiques sont utilisés pour détecter les anomalies.
ImmunoglobulinesGénétique
#2
Quels tests sont utilisés pour évaluer les chaînes légères ?
L'électrophorèse des protéines et les dosages d'immunoglobulines sont courants.
ÉlectrophorèseImmunoglobulines
#3
Les biopsies sont-elles nécessaires pour le diagnostic ?
Oui, une biopsie de moelle osseuse peut être nécessaire pour évaluer les cellules plasmatiques.
BiopsieMoelle osseuse
#4
Quels symptômes peuvent indiquer un problème avec les chaînes légères ?
Des symptômes comme des infections fréquentes ou des douleurs osseuses peuvent survenir.
InfectionsDouleur osseuse
#5
Les tests génétiques sont-ils fiables pour le diagnostic ?
Oui, les tests génétiques peuvent fournir des informations précises sur les mutations.
Tests génétiquesMutations
Symptômes
5
#1
Quels sont les symptômes d'une maladie liée aux chaînes légères ?
Les symptômes incluent fatigue, infections récurrentes et douleurs osseuses.
FatigueInfections
#2
Comment les chaînes légères affectent-elles le système immunitaire ?
Elles sont essentielles pour la production d'anticorps, influençant la réponse immunitaire.
Système immunitaireAnticorps
#3
Les troubles des chaînes légères causent-ils des symptômes neurologiques ?
Oui, des troubles peuvent entraîner des symptômes neurologiques comme des engourdissements.
Symptômes neurologiquesEngourdissement
#4
Peut-on avoir des symptômes sans anomalie génétique ?
Oui, des symptômes peuvent apparaître sans anomalies génétiques détectables.
Anomalies génétiquesSymptômes
#5
Les symptômes varient-ils selon le type de chaîne légère ?
Oui, les symptômes peuvent varier selon le type de chaîne légère impliqué.
Types de chaînes légèresSymptômes
Prévention
5
#1
Peut-on prévenir les troubles des chaînes légères ?
Il n'existe pas de méthode de prévention spécifique, mais un mode de vie sain aide.
PréventionMode de vie sain
#2
Les dépistages réguliers sont-ils recommandés ?
Des dépistages peuvent être recommandés pour les personnes à risque élevé.
DépistageRisque élevé
#3
Comment réduire le risque d'infections ?
Maintenir une bonne hygiène et se faire vacciner peut réduire le risque d'infections.
HygièneVaccination
#4
L'évitement de certaines substances est-il conseillé ?
Oui, éviter les toxines et les substances nocives peut être bénéfique.
ToxinesSubstances nocives
#5
Le soutien psychologique aide-t-il à la prévention ?
Oui, le soutien psychologique peut aider à gérer le stress et améliorer la santé globale.
Soutien psychologiqueSanté globale
Traitements
5
#1
Quels traitements sont disponibles pour les troubles des chaînes légères ?
Les traitements incluent la chimiothérapie, la thérapie ciblée et les immunothérapies.
ChimiothérapieImmunothérapie
#2
La transplantation de moelle osseuse est-elle une option ?
Oui, la transplantation de moelle osseuse peut être envisagée dans certains cas.
TransplantationMoelle osseuse
#3
Les médicaments peuvent-ils aider à gérer les symptômes ?
Oui, des médicaments peuvent être prescrits pour soulager les symptômes associés.
MédicamentsSymptômes
#4
Y a-t-il des traitements expérimentaux disponibles ?
Oui, des essais cliniques testent de nouveaux traitements pour ces troubles.
Essais cliniquesTraitements expérimentaux
#5
Comment la nutrition influence-t-elle le traitement ?
Une bonne nutrition peut soutenir le système immunitaire pendant le traitement.
NutritionSystème immunitaire
Complications
5
#1
Quelles complications peuvent survenir avec les troubles des chaînes légères ?
Les complications incluent des infections graves, des lésions organiques et des cancers.
InfectionsCancers
#2
Les troubles des chaînes légères peuvent-ils affecter les reins ?
Oui, ils peuvent entraîner des complications rénales comme la néphropathie.
ReinsNéphropathie
#3
Y a-t-il un risque accru de maladies auto-immunes ?
Oui, les patients peuvent avoir un risque accru de développer des maladies auto-immunes.
Maladies auto-immunesRisque accru
#4
Les complications cardiovasculaires sont-elles possibles ?
Oui, des complications cardiovasculaires peuvent survenir en raison de l'inflammation.
Complications cardiovasculairesInflammation
#5
Comment les complications sont-elles gérées ?
Les complications sont gérées par un suivi médical régulier et des traitements adaptés.
Suivi médicalTraitements
Facteurs de risque
5
#1
Quels sont les facteurs de risque pour les troubles des chaînes légères ?
Les facteurs incluent l'âge avancé, des antécédents familiaux et certaines maladies.
Âge avancéAntécédents familiaux
#2
Le sexe influence-t-il le risque de troubles ?
Oui, certains troubles sont plus fréquents chez les hommes que chez les femmes.
SexePrévalence
#3
Les expositions environnementales sont-elles un facteur ?
Oui, l'exposition à des produits chimiques peut augmenter le risque de troubles.
Exposition environnementaleProduits chimiques
#4
Les maladies chroniques augmentent-elles le risque ?
Oui, des maladies chroniques comme le lupus peuvent augmenter le risque.
Maladies chroniquesLupus
#5
Le mode de vie influence-t-il le risque ?
Oui, un mode de vie malsain peut contribuer à un risque accru de troubles.
Mode de vieRisque accru
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"@type": "Question",
"name": "Le soutien psychologique aide-t-il à la prévention ?",
"position": 15,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, le soutien psychologique peut aider à gérer le stress et améliorer la santé globale."
}
},
{
"@type": "Question",
"name": "Quels traitements sont disponibles pour les troubles des chaînes légères ?",
"position": 16,
"acceptedAnswer": {
"@type": "Answer",
"text": "Les traitements incluent la chimiothérapie, la thérapie ciblée et les immunothérapies."
}
},
{
"@type": "Question",
"name": "La transplantation de moelle osseuse est-elle une option ?",
"position": 17,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, la transplantation de moelle osseuse peut être envisagée dans certains cas."
}
},
{
"@type": "Question",
"name": "Les médicaments peuvent-ils aider à gérer les symptômes ?",
"position": 18,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, des médicaments peuvent être prescrits pour soulager les symptômes associés."
}
},
{
"@type": "Question",
"name": "Y a-t-il des traitements expérimentaux disponibles ?",
"position": 19,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, des essais cliniques testent de nouveaux traitements pour ces troubles."
}
},
{
"@type": "Question",
"name": "Comment la nutrition influence-t-elle le traitement ?",
"position": 20,
"acceptedAnswer": {
"@type": "Answer",
"text": "Une bonne nutrition peut soutenir le système immunitaire pendant le traitement."
}
},
{
"@type": "Question",
"name": "Quelles complications peuvent survenir avec les troubles des chaînes légères ?",
"position": 21,
"acceptedAnswer": {
"@type": "Answer",
"text": "Les complications incluent des infections graves, des lésions organiques et des cancers."
}
},
{
"@type": "Question",
"name": "Les troubles des chaînes légères peuvent-ils affecter les reins ?",
"position": 22,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, ils peuvent entraîner des complications rénales comme la néphropathie."
}
},
{
"@type": "Question",
"name": "Y a-t-il un risque accru de maladies auto-immunes ?",
"position": 23,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, les patients peuvent avoir un risque accru de développer des maladies auto-immunes."
}
},
{
"@type": "Question",
"name": "Les complications cardiovasculaires sont-elles possibles ?",
"position": 24,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, des complications cardiovasculaires peuvent survenir en raison de l'inflammation."
}
},
{
"@type": "Question",
"name": "Comment les complications sont-elles gérées ?",
"position": 25,
"acceptedAnswer": {
"@type": "Answer",
"text": "Les complications sont gérées par un suivi médical régulier et des traitements adaptés."
}
},
{
"@type": "Question",
"name": "Quels sont les facteurs de risque pour les troubles des chaînes légères ?",
"position": 26,
"acceptedAnswer": {
"@type": "Answer",
"text": "Les facteurs incluent l'âge avancé, des antécédents familiaux et certaines maladies."
}
},
{
"@type": "Question",
"name": "Le sexe influence-t-il le risque de troubles ?",
"position": 27,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, certains troubles sont plus fréquents chez les hommes que chez les femmes."
}
},
{
"@type": "Question",
"name": "Les expositions environnementales sont-elles un facteur ?",
"position": 28,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, l'exposition à des produits chimiques peut augmenter le risque de troubles."
}
},
{
"@type": "Question",
"name": "Les maladies chroniques augmentent-elles le risque ?",
"position": 29,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, des maladies chroniques comme le lupus peuvent augmenter le risque."
}
},
{
"@type": "Question",
"name": "Le mode de vie influence-t-il le risque ?",
"position": 30,
"acceptedAnswer": {
"@type": "Answer",
"text": "Oui, un mode de vie malsain peut contribuer à un risque accru de troubles."
}
}
]
}
]
}
From the Department of Clinical Therapeutics, National and Kapodistrian University of Athens, School of Medicine, Athens (E.K., M.A.D.); the Amyloidosis Research and Treatment Center, Fondazione IRCCS Policlinico San Matteo, and the Department of Molecular Medicine, University of Pavia, Pavia, Italy (G.P., G.M.); the Department of Hematology, University Medical Center Utrecht, University Utrecht, Utrecht (M.C.M.), the Amyloidosis Center of Expertise, University of Groningen, University Medical Center Groningen, Groningen (W.R.), and Janssen Research and Development, Leiden (B.T., J. Vermeulen) - all in the Netherlands; University College London, London (A.D.W.); Centre Hospitalier Universitaire (CHU) and Reference Center for AL Amyloidosis, Limoges (A.J.), Département de Néphrologie et Transplantation d'Organes, Centre de Référence des Maladies Rénales Rares, Hôpital Rangueil, CHU de Toulouse, Toulouse (A.H.), and the Department of Hematology, CHU Lille, University of Lille, Lille (S.M.) - all in France; the Department of Lymphoma and Myeloma, Division of Cancer Medicine, University of Texas M.D. Anderson Cancer Center, Houston (H.C.L.); the Amyloidosis Center, Boston University School of Medicine and Boston Medical Center (V.S.), and the Division of Hematology/Oncology, John C. Davis Myeloma and Amyloid Program, Tufts Medical Center (R.L.C.) - both in Boston; the Victorian and Tasmanian Amyloidosis Service, Department of Haematology, Monash University Eastern Health Clinical School, Melbourne, VIC (S.G.), the Department of Haematology, Princess Alexandra Hospital and University of Queensland Medical School, Brisbane (P.M.), and the Department of Clinical Haematology, Westmead Hospital, Westmead, NSW (F.K.) - all in Australia; Cross Cancer Institute, University of Alberta, Edmonton (C.P.V.), the Division of Hematology, London Health Sciences Centre, London Regional Cancer Program, Western University, London, ON (S.L.), and the Division of Hematology, Vancouver General Hospital, BC Cancer, University of British Columbia, Vancouver (K. Song) - all in Canada; Peking University People's Hospital, Peking University Institute of Hematology, National Clinical Research Center for Hematologic Disease, Collaborative Innovation Center of Hematology, Beijing (J.L.); Medical Department V (Hematology/Oncology/Rheumatology), Amyloidosis Center, Heidelberg University Hospital, Heidelberg (S.S.), and Hämatologisch-Onkologische Praxis Altona, Hamburg (T.H.) - both in Germany; the Department of Hematology, Hadassah Medical Center, Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem (M.E.G.); the Department of Hematology, Japanese Red Cross Medical Center, Tokyo (K. Suzuki), and the Department of Hematology, Japan Community Health Care Organization Kyoto Kuramaguchi Medical Center, Kyoto (C.S.) - both in Japan; the Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine (K.K.), and the Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine (J.-S.K.) - both in Seoul, South Korea; the Amyloidosis and Myeloma Unit, Hospital Clinic of Barcelona, August Pi i Sunyer Biomedical Research Institute, Barcelona (M.T.C.); the Department of Hematology, Ankara University, Ankara, Turkey (M.B.); the Division of Medical Oncology, Department of Medicine, University of Washington, Seattle (E.L.); the Department of Hematology and Medical Oncology, Taussig Cancer Center, Cleveland Clinic, Cleveland (J. Valent), and the Division of Hematology, Ohio State University Comprehensive Cancer Center, Columbus (N.B.) - both in Ohio; Clínica São Germano, São Paulo (V.H.), and Clinica CEHON, Rede D'Or Oncologia, Salvador (E.C.) - both in Brazil; the Department of Medicine, University of California, San Francisco, San Francisco (S.W.W.), the Department of Hematology and Hematopoietic Cell Transplantation, Judy and Bernard Briskin Center for Multiple Myeloma Research, City of Hope, Duarte (M.R.), and Janssen Research and Development, Los Angeles (N.T.) - all in California; the Department of Internal Medicine, Division of Hematology/Oncology, Columbia University Medical Center, New York (D.B.); the Penn Amyloidosis Program, Abramson Cancer Center, Perelman School of Medicine, University of Pennsylvania, Philadelphia (A.J.W.), and Janssen Research and Development, Spring House (X.Q., S.Y.V., B.M.W.) - both in Pennsylvania; Vanderbilt University Medical Center and Veterans Affairs Tennessee Valley Healthcare System, Nashville (S.A.G.); the Department of Oncology, Karmanos Cancer Institute, Wayne State University, Detroit (J.A.Z.); the Department of Hematology, Institute of Hematology and Transfusion Medicine, Warsaw, Poland (K.J.); and Genmab US, Princeton (T.A.), and Janssen Research and Development, Raritan (J.M.S., S.H.Z.) - both in New Jersey.
Publications dans "Gènes de chaine légère d'immunoglobuline" :
From the Department of Clinical Therapeutics, National and Kapodistrian University of Athens, School of Medicine, Athens (E.K., M.A.D.); the Amyloidosis Research and Treatment Center, Fondazione IRCCS Policlinico San Matteo, and the Department of Molecular Medicine, University of Pavia, Pavia, Italy (G.P., G.M.); the Department of Hematology, University Medical Center Utrecht, University Utrecht, Utrecht (M.C.M.), the Amyloidosis Center of Expertise, University of Groningen, University Medical Center Groningen, Groningen (W.R.), and Janssen Research and Development, Leiden (B.T., J. Vermeulen) - all in the Netherlands; University College London, London (A.D.W.); Centre Hospitalier Universitaire (CHU) and Reference Center for AL Amyloidosis, Limoges (A.J.), Département de Néphrologie et Transplantation d'Organes, Centre de Référence des Maladies Rénales Rares, Hôpital Rangueil, CHU de Toulouse, Toulouse (A.H.), and the Department of Hematology, CHU Lille, University of Lille, Lille (S.M.) - all in France; the Department of Lymphoma and Myeloma, Division of Cancer Medicine, University of Texas M.D. Anderson Cancer Center, Houston (H.C.L.); the Amyloidosis Center, Boston University School of Medicine and Boston Medical Center (V.S.), and the Division of Hematology/Oncology, John C. Davis Myeloma and Amyloid Program, Tufts Medical Center (R.L.C.) - both in Boston; the Victorian and Tasmanian Amyloidosis Service, Department of Haematology, Monash University Eastern Health Clinical School, Melbourne, VIC (S.G.), the Department of Haematology, Princess Alexandra Hospital and University of Queensland Medical School, Brisbane (P.M.), and the Department of Clinical Haematology, Westmead Hospital, Westmead, NSW (F.K.) - all in Australia; Cross Cancer Institute, University of Alberta, Edmonton (C.P.V.), the Division of Hematology, London Health Sciences Centre, London Regional Cancer Program, Western University, London, ON (S.L.), and the Division of Hematology, Vancouver General Hospital, BC Cancer, University of British Columbia, Vancouver (K. Song) - all in Canada; Peking University People's Hospital, Peking University Institute of Hematology, National Clinical Research Center for Hematologic Disease, Collaborative Innovation Center of Hematology, Beijing (J.L.); Medical Department V (Hematology/Oncology/Rheumatology), Amyloidosis Center, Heidelberg University Hospital, Heidelberg (S.S.), and Hämatologisch-Onkologische Praxis Altona, Hamburg (T.H.) - both in Germany; the Department of Hematology, Hadassah Medical Center, Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem (M.E.G.); the Department of Hematology, Japanese Red Cross Medical Center, Tokyo (K. Suzuki), and the Department of Hematology, Japan Community Health Care Organization Kyoto Kuramaguchi Medical Center, Kyoto (C.S.) - both in Japan; the Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine (K.K.), and the Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine (J.-S.K.) - both in Seoul, South Korea; the Amyloidosis and Myeloma Unit, Hospital Clinic of Barcelona, August Pi i Sunyer Biomedical Research Institute, Barcelona (M.T.C.); the Department of Hematology, Ankara University, Ankara, Turkey (M.B.); the Division of Medical Oncology, Department of Medicine, University of Washington, Seattle (E.L.); the Department of Hematology and Medical Oncology, Taussig Cancer Center, Cleveland Clinic, Cleveland (J. Valent), and the Division of Hematology, Ohio State University Comprehensive Cancer Center, Columbus (N.B.) - both in Ohio; Clínica São Germano, São Paulo (V.H.), and Clinica CEHON, Rede D'Or Oncologia, Salvador (E.C.) - both in Brazil; the Department of Medicine, University of California, San Francisco, San Francisco (S.W.W.), the Department of Hematology and Hematopoietic Cell Transplantation, Judy and Bernard Briskin Center for Multiple Myeloma Research, City of Hope, Duarte (M.R.), and Janssen Research and Development, Los Angeles (N.T.) - all in California; the Department of Internal Medicine, Division of Hematology/Oncology, Columbia University Medical Center, New York (D.B.); the Penn Amyloidosis Program, Abramson Cancer Center, Perelman School of Medicine, University of Pennsylvania, Philadelphia (A.J.W.), and Janssen Research and Development, Spring House (X.Q., S.Y.V., B.M.W.) - both in Pennsylvania; Vanderbilt University Medical Center and Veterans Affairs Tennessee Valley Healthcare System, Nashville (S.A.G.); the Department of Oncology, Karmanos Cancer Institute, Wayne State University, Detroit (J.A.Z.); the Department of Hematology, Institute of Hematology and Transfusion Medicine, Warsaw, Poland (K.J.); and Genmab US, Princeton (T.A.), and Janssen Research and Development, Raritan (J.M.S., S.H.Z.) - both in New Jersey.
Publications dans "Gènes de chaine légère d'immunoglobuline" :
Amyloidosis Research and Treatment Center, Foundation IRCCS Policlinico San Matteo, and Department of Molecular Medicine, University of Pavia , Pavia, Italy.
Publications dans "Gènes de chaine légère d'immunoglobuline" :
To describe multimodal imaging of peculiar bilateral globular subretinal deposits and acquired serous retinal detachment in patients with systemic immunoglobulin light chain deposition....
A retrospective observational case series....
We examined six eyes in three patients (one with multiple myeloma, one with membranous nephropathy, and one with immunoglobulin A nephropathy) at the Eye and ENT Hospital of Fudan University. The pati...
Fundus appearance was documented with multimodal imaging, which included fundus photography, fundus autofluorescence, spectral domain optical coherence tomography (OCT), swept-source OCT (SS-OCT), en-...
Multimodal imaging, course, and prognosis of bilateral RPE immunoglobulin light chain deposition in patients with systemic immunoglobulin light chain deposition....
Bilateral, multiple, speckled, or patchy RPE changes in the posterior fundus that corresponded to striking multifocal hyperautofluorescence on fundus autofluorescence and lumpy, globular hyperreflecti...
We have documented the characteristic features, clinical course, and prognosis of bilateral RPE immunoglobulin light chain deposition in patients with systemic immunoglobulin light chain deposition. A...
Analysis of an individual's immunoglobulin (IG) gene repertoire requires the use of high-quality germline gene reference sets. When sets only contain alleles supported by strong evidence, AIRR sequenc...
The Adaptive Immune Receptor Repertoire Community (AIRR-C) IG Reference Sets have been developed by including only human IG heavy and light chain alleles that have been confirmed by evidence from mult...
The Reference Sets include less than half the previously recognised IG alleles (e.g. just 198 IGHV sequences), and also include a number of novel alleles: 8 IGHV alleles, 2 IGKV alleles and 5 IGLV all...
Each monoclonal antibody light chain associated with AL amyloidosis has a unique sequence. Defining how these sequences lead to amyloid deposition could facilitate faster diagnosis and lead to new tre...
Light chain sequences are collected in the Boston University AL-Base repository. Monoclonal sequences from AL amyloidosis, multiple myeloma and the healthy polyclonal immune repertoire were compared t...
AL-Base now contains 2,193 monoclonal light chain sequences from plasma cell dyscrasias. Sixteen germline precursor genes were enriched in AL amyloidosis, relative to multiple myeloma and the polyclon...
Rarely-observed light chain variable genes may carry a high risk of AL amyloidosis. New approaches are needed to define sequence-associated risk factors for AL amyloidosis. AL-Base is a foundational r...
A man in his 60s, known with multiple sclerosis, presented with seizures and paresis of the left arm and leg. Brain imaging showed a white matter lesion, right parietal, which was progressive over the...
Testing of serum-free light chains kappa (κ) and lambda (λ), along with ratio (FLCR) is essential for the diagnosis and management of monoclonal gammopathies. Accurate clinical diagnosis depends upon ...
A total of 180 healthy Saudi adults were recruited. All serum samples were assayed using the Freelite reagents from the Binding Site. The variation in reference values attributable to sex, age, BMI, a...
The new RIs for free κ and FLCR were shifted to a higher side from the manufacturer-adapted RIs. Based on the SDR cutoff value (>0.4), between-sex partition RIs were not required for all analytes exce...
Locally derived RIs for free light chains and immunoglobulins analytes specific for Saudis were established after careful consideration of various factors. These RIs were more reliable than those prov...
Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend o...
Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple-green birefringence is required for the diagnosis of AL amyloidosis. Organ biopsy is not required in 85% of patients. Ver...
N-terminal pro-brain natriuretic peptide (NT-proBNP or BNP), serum troponin T(or I), and difference between involved and uninvolved immunoglobulin free light chain values are used to classify patients...
All patients with a systemic amyloid syndrome require therapy to prevent deposition of amyloid in other organs and prevent progressive organ failure. Current first-line therapy with the best outcome i...
Delayed diagnosis remains a major obstacle to initiating effective therapy prior to the development of end-stage organ failure. Trials of antibodies to deplete deposited fibrils are underway....
AL amyloidosis is a life-threatening disease caused by deposition of immunoglobulin light chains. Whilst the mechanisms underlying light chains amyloidogenesis in vivo remain unclear, several studies ...
Free light chain (FLC) assays and the ratio of κ/λ are recommended for diagnosis, prognosis and monitoring of plasma cell dyscrasias (PCD). Limited data exists on FLC clinical specificity in patients ...
We assessed the κ, λ, and κ/λ FLC ratio using the FreeLite assay and the Sebia FLC ELISA assay in 176 patients with clinical presentations of fatigue, anemia, polyclonal hypergammaglobulinemia, joint ...
For the κ/λ ratio, 68.7 % (121/176) of specimens on the FreeLite and 87.5 % (154/176) of specimens on the Sebia assay were within RI. For κ, 68.2 % (120/176) and 72.2 % (127/176) of results were outsi...
In a cohort of patients with signs and symptoms suggestive of PCDs, but ultimately diagnosed with other conditions, Sebia FLC had improved clinical specificity relative to FreeLite, if one was using a...
Determining the type of amyloid deposits is clinically important for choosing the specific therapies for cardiac amyloidosis....
A 78-year-old woman who had been experiencing fluid retention and dyspnoea on exertion for 6 months was referred to our hospital for the management of heart failure with left ventricular hypertrophy. ...
Coexistence of ATTRv and AL within cardiac amyloidosis is extremely uncommon. In situations where incongruities arise between the amyloid type determined via immunohistochemistry findings and the amyl...
Kidney light chain (AL) amyloidosis is associated with a risk of progression to kidney replacement therapy (KRT) and death. Several studies have shown that a greater reduction in proteinuria following...
To validate graded kidney response criteria and their association with kidney and overall survival (OS)....
This retrospective, multicenter cohort was conducted at 10 referral centers for amyloidosis from 2010 to 2015 and included patients with kidney AL amyloidosis that was evaluable for kidney response an...
Four kidney response categories based on the reduction in pretreatment 24-hour urine protein (24-hour UP) levels: complete response (kidCR, 24-hour UP ≤200 mg), very good partial response (kidVGPR, >6...
Cumulative incidence of progression to KRT and OS....
Seven-hundred and thirty-two patients (335 women [45.8%]) were included, with a median (IQR) age of 63 (55-69) years. The median (IQR) baseline 24-hour proteinuria and estimated glomerular filtration ...
The results of this cohort study suggest that graded kidney response criteria offers clinically and prognostically meaningful information for treating patients with kidney AL amyloidosis. The response...
