Outcome of Patients With Newly Diagnosed Systemic Light-Chain Amyloidosis Associated With Deletion of 17p

Wong, Sandy W.; Hegenbart, Ute; Palladini, Giovanni; Shah, Gunjan L.; Landau, Heather; Warner, Melissa; Toskic, Denis; Jaccard, Arnaud; Hansen, Timon; Bladé, Joan; Cibeira, MT; Kastritis, Efstathios; Dispenzieri, Angela; Wechalekar, Ashutosh; Varga, Cindy; Schönland, Stefan; Comenzo, Raymond L.

doi:10.1016/j.clml.2018.07.292

Cited by 19 publications

(15 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The latter includes serum and urine protein electrophoresis/immunofixation, serum free light-chain assay and a bone marrow biopsy to assess the presence of clonal plasma cells (or rarely a lymphoplasmacytic or a Bcell clone). Evaluation of cytogenetic abnormalities of the neoplastic cells by interphase fluorescence in-situ hybridization is important as it may have therapeutic implications [29][30][31]. Biopsy of the affected organ(s) is usually not necessary for diagnostic confirmation as amyloid deposits can be detected in the bone marrow biopsy and/or abdominal fat aspiration in nearly 90% of patients [32].…”

Section: Al Amyloidosismentioning

confidence: 99%

Systemic amyloidosis from A (AA) to T (ATTR): a review

et al. 2020

View full text Add to dashboard Cite

Systemic amyloidosis is a rare protein misfolding and deposition disorder leading to progressive organ failure. There are over 15 types of systemic amyloidosis, each caused by a different precursor protein which promotes amyloid formation and tissue deposition. Amyloidosis can be acquired or hereditary and can affect various organs, including the heart, kidneys, liver, nerves, gastrointestinal tract, lungs, muscles, skin and soft tissues. Symptoms are usually insidious and nonspecific resulting in diagnostic delay. The field of amyloidosis has seen significant improvements over the past decade in diagnostic accuracy, prognosis prediction and management. The advent of mass spectrometry-based shotgun proteomics has revolutionized amyloid typing and has led to the discovery of new amyloid types. Accurate typing of the precursor protein is of paramount importance as the type dictates a specific management approach. In this article, we review each type of systemic amyloidosis to provide the practitioner with practical tools to improve diagnosis and management of these rare disorders.

show abstract

Section: Al Amyloidosismentioning

confidence: 99%

Systemic amyloidosis from A (AA) to T (ATTR): a review

et al. 2020

View full text Add to dashboard Cite

show abstract

“…Cytogenetic analysis and its prognostic significance have been well studied in multiple myeloma (MM), a related disorder, but its utility remains relatively unknown in AL amyloidosis. 3,4 A study conducted by Bryce et al 5 in 2009 was one of the first to describe the utility of interphase fluorescence in situ hybridization (FISH) coupled to cytoplasmic staining of specific IgH (cIg-FISH) on bone marrow plasma cells, specifically identifying t (11;14) as an adverse risk factor in AL patients. Warsame et al 6 also reported on cIg-FISH abnormalities, analyzing degree of plasma cell burden and their relationship to survival and advanced cardiac disease.…”

Section: Introductionmentioning

confidence: 99%

“…Various other studies have investigated specific FISH probes and their corresponding cytogenetics abnormalities, including 1q21 gain, 17p deletion, various trisomies (chromosomes 3,7,9,15), and hyperdiploidy. [4][5][6][7][8][9][10] This will likely continue to grow as there continues to be an increase in the variety of FISH probe analyses available.…”

Section: Introductionmentioning

confidence: 99%

AL amyloidosis: The effect of fluorescent in situ hybridization abnormalities on organ involvement and survival

et al. 2020

View full text Add to dashboard Cite

show abstract

“…Myeloma high-risk cytogenetics ( t (4;14), t (14;16), del17) are rare in AL and not an unfavorable prognostic factor in AL patients treated with bortezomib [20], while more complex karyotype clones and higher rates of del17 have a slightly worse prognosis [19, 21, 25]. Gain of 1q21 is an independent adverse prognostic factor in AL amyloidosis patients treated with melphalan and dexamethasone standard chemotherapy as well as with daratumumab [23, 24].…”

Section: Prognostic Value Of Plasma Cellular Factors In Al Amyloidosismentioning

confidence: 99%

Prognosis and Staging of AL Amyloidosis

et al. 2020

Self Cite

View full text Add to dashboard Cite

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.

show abstract

Outcome of Patients With Newly Diagnosed Systemic Light-Chain Amyloidosis Associated With Deletion of 17p

Abstract: Cardiac stage, hematologic response, and del 17p percentage impact outcomes in these cases. Emphasis should be placed on optimizing supportive care and achieving a deep hematologic response.

Cited by 19 publications

References 17 publications

Systemic amyloidosis from A (AA) to T (ATTR): a review

Systemic amyloidosis from A (AA) to T (ATTR): a review

AL amyloidosis: The effect of fluorescent in situ hybridization abnormalities on organ involvement and survival

Prognosis and Staging of AL Amyloidosis

Contact Info

Product

Resources

About