Treatment patterns and outcome following initial relapse or refractory disease in patients with systemic light chain amyloidosis.
We analyzed the outcomes following initial relapse or refractory disease in systemic light chain amyloidosis (AL) and the impact of type of therapy employed.A total of 1327 patients with AL seen at Mayo Clinic within 90 days of diagnosis, between 2006 and 2015, were reviewed. The study included 366 patients experiencing a documented hematological or organ relapse or refractory disease requiring start of second line therapy. Overall survival (OS) and time to next treatment (TTNT) were calculated from start of second line treatment.The median time to require second line treatment was 16.2 months (1-93) from the start of first line therapy. At relapse, patients received proteasome inhibitors (PI; 45.1%), immunomodulators (IMiD; 22.7%), alkylators (9%), PI and IMiD combination (4.1%), autologous transplant (3.8%), steroids and other therapies (4.9%). Among these, 124 (33.9%) required change or reinstitution of therapy. The median time to require third line treatment was 31 months (95% CI; 24, 40.5) and the median overall survival (OS) was 38.8 months (95% CI; 29.6, 52.6) from the start of second line treatment. Retreatment with same therapy at relapse significantly reduced TTNT (22 m vs 32.3 m; P = .01) as compared to different therapy; but did not have any impact OS (30.8 m vs 51.1 m; P = .5). In conclusion, this study provides important information about outcomes of patients with AL who require second line treatment for relapsed/refractory disease . Treatment with a different therapy at relapse improves time to next therapy but does not impact OS.
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Nidhi
http://orcid.org/0000-0003-4008-9081
N
Tandon
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Surbhi
http://orcid.org/0000-0003-3288-7614
S
Sidana
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Morie A
MA
Gertz
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Angela
A
Dispenzieri
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Martha Q
MQ
Lacy
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Francis K
FK
Buadi
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
David
D
Dingli
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Amie L
AL
Fonder
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Miriam A
MA
Hobbs
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Suzanne R
SR
Hayman
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Wilson I
WI
Gonsalves
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Yi Lisa
YL
Hwa
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Prashant
P
Kapoor
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Robert A
RA
Kyle
Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota, USA.
Nelson
http://orcid.org/0000-0002-5651-1411
N
Leung
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Ronald S
http://orcid.org/0000-0002-8284-3495
RS
Go
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
John A
JA
Lust
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Stephen J
SJ
Russell
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Steven R
SR
Zeldenrust
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
S Vincent
SV
Rajkumar
Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.
Shaji K
SK
Kumar
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000175:diagnosis / Q000378:metabolism* / Q000401:mortality / Q000628:therapy*; D000971:Antineoplastic Combined Chemotherapy Protocols; D003131:Combined Modality Therapy; D018450:Disease Progression; D004351:Drug Resistance; D005260:Female; D005500:Follow-Up Studies; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D000075363:Immunoglobulin Light-chain Amyloidosis; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012720:Severity of Illness Index; D016019:Survival Analysis; D016896:Treatment Outcome
D007147:Immunoglobulin Light Chains