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Treatment patterns and outcome following initial relapse or refractory disease in patients with systemic light chain amyloidosis
Author(s) -
Tandon Nidhi,
Sidana Surbhi,
Gertz Morie A.,
Dispenzieri Angela,
Lacy Martha Q.,
Buadi Francis K.,
Dingli David,
Fonder Amie L.,
Hobbs Miriam A.,
Hayman Suzanne R.,
Gonsalves Wilson I.,
Hwa Yi Lisa,
Kapoor Prashant,
Kyle Robert A.,
Leung Nelson,
Go Ronald S.,
Lust John A.,
Russell Stephen J.,
Zeldenrust Steven R.,
Rajkumar S. Vincent,
Kumar Shaji K.
Publication year - 2017
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.24723
Subject(s) - amyloidosis , medicine , refractory (planetary science) , disease , systemic disease , al amyloidosis , outcome (game theory) , immunoglobulin light chain , immunology , antibody , biology , astrobiology , mathematics , mathematical economics
Abstract 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.