Seeking confidence in the diagnosis of systemic AL (Ig light-chain) amyloidosis: patients can have both monoclonal gammopathies and hereditary amyloid proteins

Comenzo, Raymond L.; Zhou, Ping; Fleisher, Martin; Clark, Bradly; Teruya‐Feldstein, Julie

doi:10.1182/blood-2005-10-4148

Cited by 135 publications

(66 citation statements)

References 25 publications

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“…In some instances, more than one possible amyloidforming protein is detected in a patient [74,113]. This is the case, e.g., of patients who carry an amyloidogenic TTR variant, who are concomitantly carriers of monoclonal gammapathy of undetermined significance (MGUS).…”

Section: Measurement Of the Amyloidogenic Precursormentioning

confidence: 98%

Biochemical markers in early diagnosis and management of systemic amyloidoses

Lavatelli

Albertini

Fonzo

et al. 2014

Clinical Chemistry and Laboratory Medicine (CCLM)

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Systemic amyloid diseases are characterized by widespread protein deposition as amyloid fibrils. Precise diagnostic framing is the prerequisite for a correct management of patients. This complex process is achieved through a series of steps, which include detection of the tissue amyloid deposits, identification of the amyloid type, demonstration of the amyloidogenic precursor, and evaluation of organ dysfunction/damage. Laboratory medicine plays a central role in the diagnosis and management of systemic amyloidoses, through the quantification of the amyloidogenic precursor and evaluation of end-organ damage using biomarkers.

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Section: Measurement Of the Amyloidogenic Precursormentioning

confidence: 98%

Biochemical markers in early diagnosis and management of systemic amyloidoses

Lavatelli

Albertini

Fonzo

et al. 2014

Clinical Chemistry and Laboratory Medicine (CCLM)

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“…If the work-up reveals a monoclonal protein, then the type is most probably AL; however, the possible coexistence of a monoclonal gammopathy and hereditary or age-related systemic amyloidosis should always be considered. 9 If the work-up does not reveal a monoclonal gammopathy, then the AL type is unlikely and evaluation for other types should commence.…”

Section: Recent Advances In Almentioning

confidence: 99%

“…11 DNA sequencing of genes related to hereditary variants is useful after the type of amyloid has been identified both as confirmatory testing for the patient and as a key screening test for the patient's kindred. 9 Immunoelectron microscopy has proven to be more reliable than immunohistochemistry for typing amyloidosis. 12 Laser microdissection with mass spectrometry (LMD/MS) is a recent advance in diagnostic testing that identifies the type of amyloid with high reliability and accuracy.…”

Section: Recent Advances In Almentioning

confidence: 99%

Consensus guidelines for the conduct and reporting of clinical trials in systemic light-chain amyloidosis

et al. 2012

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“…In these cases, systemic AL may be secondary or familial rather than light-chain derived. 8 The origin of amyloid can be confirmed by laser microdissection with mass spectrometry and is the current standard for typing the protein constituents of the amyloid deposits. 9 Immunogold staining has also been used to confirm the origin of amyloid; 10 immunohistochemistry, although routinely performed is often inaccurate.…”

Section: Introductionmentioning

confidence: 99%

“…11 Documenting the type of amyloid is essential as treatment is distinct for each type. 8,12 An algorithm outlining the workup of a patient with AL is shown in Figure 1.…”

Section: Introductionmentioning

confidence: 99%

Light-chain amyloidosis: SCT, novel agents and beyond

Rosenzweig

Giralt

Landau

2012

Bone Marrow Transplant

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Light-chain amyloidosis is a plasma cell dyscrasia characterized by the production of fibrillar proteins comprised of monoclonal light chains, which deposit in tissues causing multiorgan dysfunction and death. The diagnosis is challenging and requires a biopsy and often specialized testing to confirm the subtype of systemic disease. The goal of treatment is eradication of the monoclonal plasma cell population and suppression of the pathologic light chains, which improve organ function and extend survival. Standard treatment approaches have included high-dose melphalan followed by autologous hematopoietic SCT or oral melphalan with dexamethasone. The use of novel agents (thalidomide, lenalidomide and bortezomib) alone and in combination with steroids and alkylating agents has shown efficacy and continues to be explored. A risk-adapted approach to SCT followed by novel agents as consolidation, reduces treatment-related mortality with promising activity. Immunotherapy targeting pathologic plasma cells and amyloid fibrils is being developed and could potentially eliminate visceral amyloid deposits. Improved understanding of the biology that renders light-chains amyloidogenic and a commitment to refer patients to specialized centers conducting well-designed clinical trials is essential to improve patient outcomes.

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Seeking confidence in the diagnosis of systemic AL (Ig light-chain) amyloidosis: patients can have both monoclonal gammopathies and hereditary amyloid proteins

Abstract: Investigators in the United

Cited by 135 publications

References 25 publications

Biochemical markers in early diagnosis and management of systemic amyloidoses

Biochemical markers in early diagnosis and management of systemic amyloidoses

Consensus guidelines for the conduct and reporting of clinical trials in systemic light-chain amyloidosis

Light-chain amyloidosis: SCT, novel agents and beyond

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