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 language | English (US) |
|---|---|
| Journal | British journal of haematology |
| DOIs | |
| State | Published - Jan 1 2019 |
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Keywords
- free light chains
- heart
- kidney
- light chain amyloidosis
- survival
ASJC Scopus subject areas
- Hematology
Cite this
Clinical features, laboratory characteristics and outcomes of patients with renal versus cardiac light chain amyloidosis. / Sidana, Surbhi; Tandon, Nidhi; Gertz, Morie; Dispenzieri, Angela; Ramirez-Alvarado, Marina; Murray, David L.; Kourelis, Taxiarchis; Buadi, Francis K.; Kapoor, Prashant; Gonsalves, Wilson; Warsame, Rahma; Lacy, Martha; Kyle, Robert A.; Rajkumar, S Vincent; Kumar, Shaji K; Leung, Nelson.
In: British journal of haematology, 01.01.2019.Research output: Contribution to journal › Article
}
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
UR - http://www.scopus.com/inward/record.url?scp=85062514029&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85062514029&partnerID=8YFLogxK
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
ER -