Definition of Organ Involvement and Treatment Response in Primary Systemic Amyloidosis (AL): A Consensus Opinion from the 10TH International Symposium on Amyloid and Amyloidosis

Gertz, Morie A.; Comenzo, Ray; Falk, Rodney H.; Fermand, Jean Paul; Hazenberg, Bouke P. C.; Hawkins, Philip N.; Merlini, Giampaolo; Moreau, Philippe; Ronco, Pierre; Sanchorawala, Vaishali; Sezer, Orhan; Solomon, Alan; Grateau, G

doi:10.1201/9781420037494-53

Cited by 7 publications

(9 citation statements)

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“…Assessment included clinical examination, detailed blood and urine analysis (including assessment of serum and urine monoclonal Ig and serum free light chains), serial 123 I‐labelled serum amyloid P component (SAP) scintigraphy to assess whole body amyloid load, electrocardiogram (ECG) and echocardiogram. Organ involvement was defined according to the international amyloidosis consensus criteria (Gertz et al , ). Patients were treated with chemotherapy regimens used to treat myeloma during the study period.…”

Section: Methodsmentioning

confidence: 99%

“…Survival studies were performed on 163 patients with available follow‐up data (median follow‐up 35 months (2·4–85·3 months). Cardiac involvement for survival studies was defined according to the international amyloidosis consensus criteria (Gertz et al , ) and/or N‐terminal prohormone of brain natriuretic peptide (NT‐proBNP) >332 ng/l. There were 108 deaths, of which 8 were due to infection and 89 due to amyloidosis.…”

Section: Methodsmentioning

confidence: 99%

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Immunoparesis defined by heavy+light chain suppression is a novel marker of long‐term outcomes in cardiac AL amyloidosis

Sachchithanantham

Berlanga²,

Alvi³

et al. 2017

Br J Haematol

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Cardiac involvement and presenting dFLC (difference between involved and uninvolved free light chains) are independent predictors of outcome in systemic AL amyloidosis. These markers have limited prognostic utility in patients surviving the initial months following diagnosis. Here we assessed immunoparesis, as determined by novel heavy+light chain (HLC) immunoassays, as a prognostic marker for survival in AL amyloidosis. HLC measurements identified immunoparesis of at least one immunoglobulin (Ig) isotype in 145 (85%) patients; and severe immunoparesis (≥2 Ig isotypes suppressed by >50% below normal levels) in 29 (17%) patients. Median overall survival (OS) on intention to treat (ITT) analysis was 26·2 months. In the ITT cohort, dFLC >180 mg/l was associated with shorter OS (P = 0·05); whereas HLC immunoparesis was not prognostic. On a landmark analysis of 127 patients alive at 6 months, presenting dFLC was not prognostic for OS (P = 0·33) and severe HLC immunoparesis trended towards poorer survival (20·2 vs. 42·8 months; P = 0·09). In the subset of patients with cardiac involvement, severe HLC immunoparesis conferred very poor outcome (median OS 8·8 vs. 29·9 months, P = 0·007). In conclusion, severe HLC immunoparesis is an independent marker of long-term poor prognosis in AL patients with cardiac involvement. The pathophysiological significance of this observation needs further study.

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Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Immunoparesis defined by heavy+light chain suppression is a novel marker of long‐term outcomes in cardiac AL amyloidosis

Sachchithanantham

Berlanga²,

Alvi³

et al. 2017

Br J Haematol

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“…[4][5][6] When to suspect amyloidosis Amyloidosis can affect any organ in the body and often presents with a range of nonspecific symptoms (Table 2). 7 The initial presentation may be fatigue or weight loss, but, as the disease progresses, symptoms may come to reflect dysfunction of involved organs (Fig. 1).…”

Section: Pathogenesis Of Amyloid Depositionmentioning

confidence: 99%

Current approaches to the diagnosis and management of amyloidosis

Taylor

Sidiqi

Hare

et al. 2022

Internal Medicine Journal

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Amyloidosis is a collection of diseases caused by the misfolding of proteins that aggregate into insoluble amyloid fibrils and deposit in tissues. While these fibrils may aggregate to form insignificant localised deposits, they can also accumulate in multiple organs to the extent that amyloidosis can be an immediately life‐threatening disease, requiring urgent treatment. Recent advances in diagnostic techniques and therapies are dramatically changing the disease landscape and patient prognosis. Delays in diagnosis and treatment remain the greatest challenge, necessitating physician awareness of the common clinical presentations that suggest amyloidosis. The most common types are transthyretin (ATTR) amyloidosis followed by immunoglobulin light‐chain (AL) amyloidosis. While systemic AL amyloidosis was previously considered a death sentence with no effective therapies, significant improvement in patient survival has occurred over the past 2 decades, driven by greater understanding of the disease process, risk‐adapted adoption of myeloma therapies such as proteosome inhibitors (bortezomib) and monoclonal antibodies (daratumumab) and improved supportive care. ATTR amyloidosis is an underdiagnosed cause of heart failure. Technetium scintigraphy has made noninvasive diagnosis much easier, and ATTR is now recognised as the most common type of amyloidosis because of the increased identification of age‐related ATTR. There are emerging ATTR treatments that slow disease progression, decrease patient hospitalisations and improve patient quality of life and survival. This review aims to update physicians on recent developments in amyloidosis diagnosis and management and to provide a diagnostic and treatment framework to improve the management of patients with all forms of amyloidosis.

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“…Therapieansprechens. [17,59] Komplette hämatologische Remission (CR): CR liegt vor, wenn die Plasmazellerkrankung im Knochenmark nicht nachgewiesen werden kann (weniger als 5% Plasmazellen und kein Nachweis einer klonalen Dominanz der κoder λ-Leichtketten) und wenn gleichzeitig die monoklonale Gammopathie im Serum und Urin nicht nachgewiesen werden kann (unauffällige Proteinelektrophorese, negative Immunfixation im Serum und Urin, unauffällige Ratio der freien κund λ-Leichtketten ).…”

Section: Hämatologische Und Organbezogene Remissionskriterien Zur Erfassung Desunclassified

“…Die 50%ige Reduktion der freien Leichtketten im Serum wird als partielle Remission gewertet, wenn die initiale Konzentration über 10 mg/dl lag. Klinisches Ansprechen: Klinisches Ansprechen wird für den "performance status" nach WHO und für jedes Organ gesondert betrachtet [59].…”

Section: Partielle Hämatologische Remission (Pr)unclassified

Interdisziplinäre Leitlinien zur Diagnostik und Therapie der extrazerebralen Amyloidosen

Röcken

Ernst²,

Hund³

et al. 2006

Dtsch med Wochenschr

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Within the past 10 years, a new range of knowledge has been achieved in the field of amyloidosis, especially with regard to pathogenesis, diagnosis and therapy. Amyloidosis leads to variable and distinct symptoms and is caused by different underlying conditions. Some amyloidoses are acquired secondary to a chronic condition; others are caused by genetic mutations. Amyloid and amyloidosis occur more frequently than they are perceived. Among the frequent localized forms are the cerebral amyloidosis linked to Alzheimer disease (AD) and the pancreatic amyloidosis linked to diabetes mellitus. Among the most frequent systemic (extracerebral) forms is AL amyloidosis, which often has a poor prognosis and if untreated can rapidly lead to death. Systemic amyloidosis that happen at infancy are mainly AA amyloidosis that can progress to death already at early or at middle adulthood. Amyloidosis can be treated but therapeutic success significantly depends upon early diagnosis and proper classification of the amyloid type. It is mandatory that differential diagnosis demonstrate the presence of amyloid and clearly identify the type of the disease. Development of methods and techniques have contributed to improvements in the diagnosis and treatment. Early diagnosis and proper classification of amyloid is decisive for therapeutic options and upon them depend quality of life and mortality. The therapeutic spectrum is various and includes organ transplantation, chemotherapy, and anti-inflammatory strategies. Gene therapy and biological active substances have to be considered in the near future.

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Definition of Organ Involvement and Treatment Response in Primary Systemic Amyloidosis (AL): A Consensus Opinion from the 10TH International Symposium on Amyloid and Amyloidosis

Cited by 7 publications

References 0 publications

Immunoparesis defined by heavy+light chain suppression is a novel marker of long‐term outcomes in cardiac AL amyloidosis

Immunoparesis defined by heavy+light chain suppression is a novel marker of long‐term outcomes in cardiac AL amyloidosis

Current approaches to the diagnosis and management of amyloidosis

Interdisziplinäre Leitlinien zur Diagnostik und Therapie der extrazerebralen Amyloidosen

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