Immunoparesis status in immunoglobulin light chain amyloidosis at diagnosis affects response and survival by regimen type

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Immunoparesis status in immunoglobulin light chain amyloidosis at diagnosis affects response and survival by regimen type.

Clinical tools to guide in the appropriate treatment selection in immunoglobulin light chain (AL) amyloidosis are not well developed. We evaluated the response and outcome for various regimens at first-line treatment (n=681) and first progression (n=240) stratified by the immunoparesis status at diagnosis. Immunoparesis was assessed by the average relative difference of the uninvolved immunoglo...

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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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Nutritional status of outpatients with systemic immunoglobulin light-chain amyloidosis

Background: Maintenance of a good nutritional status is associated with prolonged survival in many chronic diseases. To date, the nutritional status of outpatients with immunoglobulin light-chain (AL) amyloidosis has not been evaluated. Objective: The aims of this study were to obtain information regarding the nutritional status of AL amyloidosis outpatients and to investigate its prognostic ro...

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Hereditary systemic immunoglobulin light-chain amyloidosis.

Several members of a family died from renal failure as a result of systemic amyloidosis. Extensive studies to detect previously documented gene mutations associated with amyloidosis failed to identify a causative factor. In search of the genetic basis for this syndrome, amyloid fibrils were isolated from renal tissue of a member of the kin who died while on renal dialysis. Amino acid sequencing...

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ژورنال

عنوان ژورنال: Haematologica

سال: 2016

ISSN: 0390-6078,1592-8721

DOI: 10.3324/haematol.2016.147041