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2018 ; 2
(7
): 769-776
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gab.com Text
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Light chain type predicts organ involvement and survival in AL amyloidosis
patients receiving stem cell transplantation
#MMPMID29615413
Sidiqi MH
; Aljama MA
; Muchtar E
; Buadi FK
; Warsame R
; Lacy MQ
; Dispenzieri A
; Dingli D
; Leung N
; Gonsalves WI
; Kumar SK
; Kapoor P
; Kourelis TV
; Hogan WJ
; Gertz MA
Blood Adv
2018[Apr]; 2
(7
): 769-776
PMID29615413
show ga
We evaluated the impact of light chain type, lambda (?) or kappa (?), on disease
features and outcomes in patients with immunoglobulin light chain (AL)
amyloidosis receiving stem cell transplant at the Mayo Clinic between October
2002 and August 2016. Patients with ? AL amyloidosis had higher rates of renal
and neurological involvement (? 69% vs ? 57%, P = .02 and ? 16% vs ? 9%, P = .03,
respectively). Patients with ? AL amyloidosis had more hepatic involvement (? 7%
vs ? 18%, P = .0003). Complete response rate was 43% for both groups and overall
response rates were similar (? 85% vs ? 91%, P = .12). Patients with ? light
chain amyloidosis had better progression-free and overall survival (PFS: ? 74
months vs ? 101 months, P = .0064 and OS: ? 121 months vs ? not reached, P =
.003). Mayo stage 2004 was more predictive of survival in the ? cohort (median OS
of 143 months stage I vs 77 months stage II vs 33 months stage III, P < .0001)
than in the ? cohort (median OS not reached for stage I and II and 102 months for
stage III, P = .044). Conditioning dose predicted survival in the ? cohort only
(median OS 149 months for melphalan 200 mg/m(2) vs 50 months for melphalan <200
mg/m(2), P < .0001; median OS ? not reached for melphalan 200 mg/m(2) or <200
mg/m(2), P = .38). On multivariate analysis, light chain type remained an
independent predictor of survival. Light chain type predicts organ involvement
and survival in patients with AL amyloidosis receiving stem cell transplant.