Systemic amyloidosis: moving into the spotlight

Cohen, Oliver; Wechalekar, Ashutosh

doi:10.1038/s41375-020-0802-4

Cited by 22 publications

(25 citation statements)

References 103 publications

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“…La amiloidosis sistémica se caracteriza por el plegamiento incorrecto de las proteínas autólogas, que se agregan en una forma fibrilar anormal y se depositan en los órganos, lo que conduce a una disfunción progresiva. 6 Todos los pacientes con amiloidosis sistémica tienen una discrasia subyacente de células plasmáticas. 6 En AL, los niveles altos de una cadena ligera de inmunoglobulina monoclonal (Ig) son producidos por una población de células plasmáticas monoclonales, asociada con mieloma múltiple franco o latente en 10 a 20%.…”

Section: Wwwmedigraphicorgmxunclassified

“…6 Todos los pacientes con amiloidosis sistémica tienen una discrasia subyacente de células plasmáticas. 6 En AL, los niveles altos de una cadena ligera de inmunoglobulina monoclonal (Ig) son producidos por una población de células plasmáticas monoclonales, asociada con mieloma múltiple franco o latente en 10 a 20%. 7 La amiloidosis de cadenas ligeras lambda es el subtipo más común en amiloidosis cutánea asociada con MM.…”

Section: Wwwmedigraphicorgmxunclassified

“…El diagnóstico de amiloidosis se realiza histológicamente mediante la demostración del depósito de amiloide. 6 Por lo tanto, es necesaria la realización de una biopsia. 12 Rubinow y cols.…”

Section: Diagnósticounclassified

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Manifestaciones dermatológicas en amiloidosis secundaria a mieloma múltiple

Ramos¹,

Rivero²,

Santos³

2021

Acta Médica Grupo Ángeles

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El mieloma múltiple (MM) es una enfermedad maligna de células plasmáticas de linfocitos; B. Bluefarb estimó que aproximadamente el 15% de los pacientes con MM desarrollarán alguna forma de amiloidosis. El término amiloidosis hace referencia a la acumulación extracelular de un material amiloide, debido a un mal plegamiento de las proteínas autólogas. El subtipo de amiloidosis de cadenas ligeras lambda (AL) es el más común en amiloidosis cutánea asociada con MM. En México, la frecuencia de amiloidosis cutánea en la consulta dermatológica es de aproximadamente 0.5% y predomina en mujeres. La infiltración de tejido por estos depósitos de amiloide se produce de forma localizada o sistémica. Cuando la amiloidosis afecta la piel, puede ser como una manifestación de amiloidosis sistémica, o una amiloidosis cutánea localizada. Las enfermedades amiloides cutáneas pueden tener manifestaciones heterogéneas, dependiendo de la ubicación del depósito amiloide dentro de la dermis o la epidermis. El diagnóstico de amiloidosis se realiza histológicamente mediante la demostración del depósito de amiloide. El tratamiento de la amiloidosis va enfocado a cada tipo. Los pacientes con mieloma asociado con amiloidosis AL con afectación de la piel tienen el peor pronóstico.

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Section: Wwwmedigraphicorgmxunclassified

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Manifestaciones dermatológicas en amiloidosis secundaria a mieloma múltiple

Ramos¹,

Rivero²,

Santos³

2021

Acta Médica Grupo Ángeles

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“…As reviewed recently, there is an increasing pool of novel organ biomarkers with potential prognostic value: growth differentiation factor-15, proadrenomedullin, osteopontin, hepatocyte growth factor, soluble suppression of tumorgenicity 2, von Willebrand factor antigen, and osteoprotegerin [71]. The link between amyloidogenic organ damage and biomarker elevation is not yet fully resolved for many of these novel biomarkers and validation of the prognostic value in large case series is warranted.…”

Section: Prognostic Value Of Organ Biomarkers In Al Amyloidosis and Smentioning

confidence: 99%

Prognosis and Staging of AL Amyloidosis

et al. 2020

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The treatment options for systemic light chain amyloidosis (AL) are currently widening in an unprecedented way, brought about by an expanding arsenal of anti-myeloma therapy as well as by novel approaches to target toxic light chains and, most recently, deposited amyloid directly. In this context, accurate estimates of prognosis in AL, which allow for reliable patient advice and for example comparison of different therapies, are particularly important to clinicians. Some biomarkers and especially the genetic background of the underlying clonal disease as evaluated by interphase fluorescence in situ hybridization even have predictive value, enabling an appropriate treatment selection. Derived from the most frequently involved organs in AL, heart and kidney, this review focuses on overall survival and renal survival. A comprehensive overview and summary of reported prognostic factors and biomarkers in AL is given and the most important and validated factors are highlighted. Finally, established staging systems in AL as well as validated and perspective response criteria are presented.

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“…Подоб ный «прецедент» хорошо известен в онкогематоло гии -это системный ALамилоидоз, тяжелое заболе вание с минимальным клоном плазмоцитов в костном мозге, но крайне неблагоприятным в отсутствие лече ния прогнозом, которое длительное время являлось предметом неразрешимых противоречий между гемато логами и нефрологами. Достаточно давно разработаны и применяются, в том числе и в России, эффективные схемы химиотерапии ALамилоидоза, направленные на элиминацию опухолевого клона [42][43][44][45][46][47]. Такая же лечебная стратегия должна быть применена и при не амилоидных формах поражения почек, ассоциирован ных с МГ [6,[13][14][15][48][49][50].…”

Section: комментарийunclassified

Kidney involvement in monoclonal gammopathies: multidisciplinary approach in oncohematology and nephrology

et al. 2020

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The pathogenetic relationship of kidney damage and an aberrant clone of the B-cell line producing nephrotoxic monoclonal immunoglobulin underlies the concept of “monoclonal gammopathy of renal significance” (MGRS). Herein the aberrant clone does not reach the criteria necessary for initiating antitumor therapy according to oncohematological indications. MGRS is a new nosology in modern nephrology and oncohematology. Monoclonal protein’s pathological effects on kidney parenchyma result in irreversible decline of kidney function till the end stage renal disease that in line with the position of International Consensus of hematologists and nephrologists determinates critical necessity for clone specific treatment in patients with MGRS despite the absence of hematological indications for treatment initiation. Main challenge of MGRS in Russian Federation is an inaccessibility of an in-time diagnostic and appropriate treatment for the great majority of patients due to the following reasons: i) limited knowledge about the MGRS among hematologists and nephrologists; ii) lack of necessary diagnostic resources in most health-care facilities; iii) lack of approved clinical recommendations and medical economic standards for treatment of this pathological entity. In order to overcome these limitations, leading oncohematologists and nephrologists of the Russian Federation on behalf of professional communities at the end of 2019 published a conciliation document: “Monoclonal gammopathy of renal significance: Consensus of hematologists and nephrologists of Russia on the establishment of nosology, diagnostic approach and rationale for clone specific treatment”. Consensus document comprises the opinion of experts – leading nephrologists and hematologists of Russian Federation – on the problem of MGRS including the incoherence in nosology classification, diagnostics approach and rationale for clone specific treatment. Consensus document is based on conclusions and agreements reached during the conference of leading nephrologists and hematologists of Russia which was held in the framework of symposia “Plasma cell dyscrasias and lymphoproliferative diseases: modern approaches to therapy”, 15–16 of March 2019, Pavlov First Saint Petersburg State Medical University, Saint Petersburg, Russia. Consensus is intended to define the principal practical steps to resolve the problem of MGRS in Russian Federation that are summarized as final clauses which we present here.

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Systemic amyloidosis: moving into the spotlight

Cited by 22 publications

References 103 publications

Manifestaciones dermatológicas en amiloidosis secundaria a mieloma múltiple

Manifestaciones dermatológicas en amiloidosis secundaria a mieloma múltiple

Prognosis and Staging of AL Amyloidosis

Kidney involvement in monoclonal gammopathies: multidisciplinary approach in oncohematology and nephrology

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