Clinical features, laboratory characteristics and outcomes of patients with renal versus cardiac light chain amyloidosis

Surbhi Sidana, Nidhi Tandon, Morie Gertz, Angela Dispenzieri, Marina Ramirez-Alvarado, David L. Murray, Taxiarchis Kourelis, Francis K. Buadi, Prashant Kapoor, Wilson Gonsalves, Rahma Warsame, Martha Lacy, Robert A. Kyle, S Vincent Rajkumar, Shaji K Kumar, Nelson Leung

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1 Citation (Scopus)

Abstract

This study evaluated the differences in clinical features of 1077 newly diagnosed AL amyloidosis patients with renal involvement (n = 229, 21%), both cardiac and renal involvement (n = 443, 41%) and cardiac involvement (n = 405, 38%). Significant differences in dFLC (difference in involved and uninvolved light chains) were noted (renal, both, cardiac median: 83, 234 and 349 mg/l, P < 0.001). The proportion of patients with ≥ 10% bone marrow plasma cells (BMPCs) was lowest in renal only patients: 44%, 57%, 64%, respectively, P < 0.001. In a multivariate linear regression model incorporating organ involvement type and BMPCs ≥10%, organ involvement was a significant predictor of dFLC (P < 0.001). Median overall survival (OS) across the three groups was 83 vs. 19 vs. 16 months (P < 0.001) in patients not undergoing transplant and 5-year OS in patients undergoing transplant was 90% vs. 75% vs. 64% (P = 0.007), respectively. In conclusion, renal involvement alone or renal + cardiac involvement in AL amyloidosis is associated with lower circulating light chain burden, which cannot be fully explained by BMPC burden alone. Increased sensitivity of the kidney to light chains, given significant interactions with the renal tubular system and secretion of modified light chain products may play a role in pathogenesis of renal AL amyloidosis and warrants further investigation.

Original languageEnglish (US)
JournalBritish journal of haematology
DOIs
StatePublished - Jan 1 2019

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Amyloidosis
Kidney
Light
Plasma Cells
Bone Marrow Cells
Linear Models
Transplants
Survival

Keywords

  • free light chains
  • heart
  • kidney
  • light chain amyloidosis
  • survival

ASJC Scopus subject areas

  • Hematology

Cite this

@article{9316056f821047ea8f06d56e3a895fe8,
title = "Clinical features, laboratory characteristics and outcomes of patients with renal versus cardiac light chain amyloidosis",
abstract = "This study evaluated the differences in clinical features of 1077 newly diagnosed AL amyloidosis patients with renal involvement (n = 229, 21{\%}), both cardiac and renal involvement (n = 443, 41{\%}) and cardiac involvement (n = 405, 38{\%}). Significant differences in dFLC (difference in involved and uninvolved light chains) were noted (renal, both, cardiac median: 83, 234 and 349 mg/l, P < 0.001). The proportion of patients with ≥ 10{\%} bone marrow plasma cells (BMPCs) was lowest in renal only patients: 44{\%}, 57{\%}, 64{\%}, respectively, P < 0.001. In a multivariate linear regression model incorporating organ involvement type and BMPCs ≥10{\%}, organ involvement was a significant predictor of dFLC (P < 0.001). Median overall survival (OS) across the three groups was 83 vs. 19 vs. 16 months (P < 0.001) in patients not undergoing transplant and 5-year OS in patients undergoing transplant was 90{\%} vs. 75{\%} vs. 64{\%} (P = 0.007), respectively. In conclusion, renal involvement alone or renal + cardiac involvement in AL amyloidosis is associated with lower circulating light chain burden, which cannot be fully explained by BMPC burden alone. Increased sensitivity of the kidney to light chains, given significant interactions with the renal tubular system and secretion of modified light chain products may play a role in pathogenesis of renal AL amyloidosis and warrants further investigation.",
keywords = "free light chains, heart, kidney, light chain amyloidosis, survival",
author = "Surbhi Sidana and Nidhi Tandon and Morie Gertz and Angela Dispenzieri and Marina Ramirez-Alvarado and Murray, {David L.} and Taxiarchis Kourelis and Buadi, {Francis K.} and Prashant Kapoor and Wilson Gonsalves and Rahma Warsame and Martha Lacy and Kyle, {Robert A.} and Rajkumar, {S Vincent} and Kumar, {Shaji K} and Nelson Leung",
year = "2019",
month = "1",
day = "1",
doi = "10.1111/bjh.15832",
language = "English (US)",
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TY - JOUR

T1 - Clinical features, laboratory characteristics and outcomes of patients with renal versus cardiac light chain amyloidosis

AU - Sidana, Surbhi

AU - Tandon, Nidhi

AU - Gertz, Morie

AU - Dispenzieri, Angela

AU - Ramirez-Alvarado, Marina

AU - Murray, David L.

AU - Kourelis, Taxiarchis

AU - Buadi, Francis K.

AU - Kapoor, Prashant

AU - Gonsalves, Wilson

AU - Warsame, Rahma

AU - Lacy, Martha

AU - Kyle, Robert A.

AU - Rajkumar, S Vincent

AU - Kumar, Shaji K

AU - Leung, Nelson

PY - 2019/1/1

Y1 - 2019/1/1

N2 - This study evaluated the differences in clinical features of 1077 newly diagnosed AL amyloidosis patients with renal involvement (n = 229, 21%), both cardiac and renal involvement (n = 443, 41%) and cardiac involvement (n = 405, 38%). Significant differences in dFLC (difference in involved and uninvolved light chains) were noted (renal, both, cardiac median: 83, 234 and 349 mg/l, P < 0.001). The proportion of patients with ≥ 10% bone marrow plasma cells (BMPCs) was lowest in renal only patients: 44%, 57%, 64%, respectively, P < 0.001. In a multivariate linear regression model incorporating organ involvement type and BMPCs ≥10%, organ involvement was a significant predictor of dFLC (P < 0.001). Median overall survival (OS) across the three groups was 83 vs. 19 vs. 16 months (P < 0.001) in patients not undergoing transplant and 5-year OS in patients undergoing transplant was 90% vs. 75% vs. 64% (P = 0.007), respectively. In conclusion, renal involvement alone or renal + cardiac involvement in AL amyloidosis is associated with lower circulating light chain burden, which cannot be fully explained by BMPC burden alone. Increased sensitivity of the kidney to light chains, given significant interactions with the renal tubular system and secretion of modified light chain products may play a role in pathogenesis of renal AL amyloidosis and warrants further investigation.

AB - This study evaluated the differences in clinical features of 1077 newly diagnosed AL amyloidosis patients with renal involvement (n = 229, 21%), both cardiac and renal involvement (n = 443, 41%) and cardiac involvement (n = 405, 38%). Significant differences in dFLC (difference in involved and uninvolved light chains) were noted (renal, both, cardiac median: 83, 234 and 349 mg/l, P < 0.001). The proportion of patients with ≥ 10% bone marrow plasma cells (BMPCs) was lowest in renal only patients: 44%, 57%, 64%, respectively, P < 0.001. In a multivariate linear regression model incorporating organ involvement type and BMPCs ≥10%, organ involvement was a significant predictor of dFLC (P < 0.001). Median overall survival (OS) across the three groups was 83 vs. 19 vs. 16 months (P < 0.001) in patients not undergoing transplant and 5-year OS in patients undergoing transplant was 90% vs. 75% vs. 64% (P = 0.007), respectively. In conclusion, renal involvement alone or renal + cardiac involvement in AL amyloidosis is associated with lower circulating light chain burden, which cannot be fully explained by BMPC burden alone. Increased sensitivity of the kidney to light chains, given significant interactions with the renal tubular system and secretion of modified light chain products may play a role in pathogenesis of renal AL amyloidosis and warrants further investigation.

KW - free light chains

KW - heart

KW - kidney

KW - light chain amyloidosis

KW - survival

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U2 - 10.1111/bjh.15832

DO - 10.1111/bjh.15832

M3 - Article

C2 - 30836444

AN - SCOPUS:85062514029

JO - British Journal of Haematology

JF - British Journal of Haematology

SN - 0007-1048

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