Predicting survival in light chain amyloidosis

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چکیده

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T1 mapping and survival in systemic light-chain amyloidosis

AIMS To assess the prognostic value of myocardial pre-contrast T1 and extracellular volume (ECV) in systemic amyloid light-chain (AL) amyloidosis using cardiovascular magnetic resonance (CMR) T1 mapping. METHODS AND RESULTS One hundred patients underwent CMR and T1 mapping pre- and post-contrast. Myocardial ECV was calculated at contrast equilibrium (ECV(i)) and 15 min post-bolus (ECVb). Fift...

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Light Chain Amyloidosis

Light chain (AL) amyloidosis is caused by a usually small plasma-cell clone that is able to produce the amyloidogenic light chains. They are able to misfold and aggregate, deposit in tissues in the form of amyloid fibrils and lead to irreversible organ dysfunction and eventually death if treatment is late or ineffective. Cardiac damage is the most important prognostic determinant. The risk of d...

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Atypical immunoglobulin light chain amyloidosis

BACKGROUND Primary immunoglobulin light chain amyloidosis (AL amyloidosis) is a plasma cell disorder which mainly affects heart, kidneys, liver, and peripheral nervous system. Cases of atypical AL amyloidosis presented as spontaneous vertebral compression fractures have been rarely reported, and data about the management and clinical outcomes of the patients are scarce. METHODS Herein, we pre...

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Light-chain (AL) amyloidosis: diagnosis and treatment.

Light-chain (AL) amyloidosis is the most common form of systemic amyloidosis and is associated with an underlying plasma cell dyscrasia. The disease often is difficult to recognize because of its broad range of manifestations and what often are vague symptoms. The clinical syndromes at presentation include nephrotic-range proteinuria with or without renal dysfunction, hepatomegaly, congestive h...

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Systemic Light Chain Amyloidosis Mimicking Rheumatic Disorders

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ژورنال

عنوان ژورنال: Haematologica

سال: 2019

ISSN: 0390-6078,1592-8721

DOI: 10.3324/haematol.2019.218859