Kinetics of organ response and survival following normalization of the serum free light chain ratio in AL amyloidosis.
Despite successful treatment of the clonal plasma cell implicated in its pathogenesis, patients with AL amyloidosis (AL) experience significant morbidity related to underlying amyloid mediated organ dysfunction. While normalization of the serum free light chain measurements [normal ratio of involved and uninvolved free light chains (nFLCr)] is the goal of therapy and centerpiece of hematologic response criteria, achieving (or not achieving) meaningful organ response (OR) is clinically significant for its implications on long-term symptomatology as well as overall survival (OS), and remains the ultimate goal of treatment. Expectations for organ recovery following successful therapy leading to nFLCr in AL remain poorly described. We evaluated the timeframe and predictive factors for OR, and long-term outcome, in 313 AL patients who achieved nFLCr following therapy initiation. OR was seen in 80% of surviving AL patients within 1-year of nFLCr. Patients achieving early OR within 1 year of nFLCr had superior OS compared with those who despite obtaining nFLCr did not achieve early OR. We further evaluated factors predicting OR and OS among patients achieving nFLCr. Higher values of dFLC (involved-uninvolved) at diagnosis predict OR, and early OR predicts improved OS following successful hematologic therapy in AL.
Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota.
Gregory P
GP
Kaufman
Angela
A
Dispenzieri
Morie A
MA
Gertz
Martha Q
MQ
Lacy
Francis K
FK
Buadi
Suzanne R
SR
Hayman
Nelson
N
Leung
David
D
Dingli
John A
JA
Lust
Yi
Y
Lin
Prashant
P
Kapoor
Ronald S
RS
Go
Steven R
SR
Zeldenrust
Robert A
RA
Kyle
S Vincent
SV
Rajkumar
Shaji K
SK
Kumar
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000276:immunology / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D003907:Dexamethasone / Q000627:therapeutic use; D016903:Drug Monitoring; D005260:Female; D006321:Heart / Q000187:drug effects*; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000097:blood*; D007668:Kidney / Q000187:drug effects* / Q000276:immunology / Q000473:pathology; D008099:Liver / Q000187:drug effects* / Q000276:immunology / Q000473:pathology; D008297:Male; D008558:Melphalan / Q000627:therapeutic use; D008875:Middle Aged; D010950:Plasma Cells / Q000187:drug effects / Q000276:immunology / Q000473:pathology; D061988:Proteasome Inhibitors / Q000627:therapeutic use; D012189:Retrospective Studies; D016019:Survival Analysis; D014182:Transplantation, Autologous
D007147:Immunoglobulin Light Chains; D061988:Proteasome Inhibitors; D003907:Dexamethasone; D008558:Melphalan