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Ten‐year survivors in AL amyloidosis: characteristics and treatment pattern
Author(s) -
Muchtar Eli,
Gertz Morie A.,
Lacy Martha Q.,
Go Ronald S.,
Buadi Francis K.,
Dingli David,
Grogan Martha,
AbouEzzeddine Omar F.,
Hayman Suzanne R.,
Kapoor Prashant,
Leung Nelson,
Fonder Amie,
Hobbs Miriam,
Hwa Yi Lisa,
Gonsalves Wilson,
Warsame Rahma,
Kourelis Taxiarchis V.,
Russell Stephen,
Lust John A.,
Lin Yi,
Zeldenrust Steven,
Kyle Robert A.,
Rajkumar S. Vincent,
Kumar Shaji K.,
Dispenzieri Angela
Publication year - 2019
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.16096
Subject(s) - interquartile range , medicine , multivariate analysis , al amyloidosis , population , amyloidosis , surgery , immunology , environmental health , antibody , immunoglobulin light chain
Summary Improvement in survival in Light chain (AL) amyloidosis has been seen over recent decades, enabling more patients to achieve long‐term survival. Patients with AL amyloidosis who survived ≥10 years from time of diagnosis ( n = 186) were the subject of this study. Ten‐year survivors represented 22% of the total population. These patients were characterized by favourable patient, organ and plasma cell features. Of note, trisomies were less common among 10‐year survivors compared to those who did not survive to 10 years. All‐time best haematological response was complete response in 67%, very good partial response in 30%, partial response in 2% and no response in 1%, with 11% having received a consolidative strategy for inadequate response to first line therapy. The overall organ response rate to first‐line therapy was 76%, which increased to 86% when considering subsequent line(s) of therapy. Forty‐seven percent of the 10‐year survivors did not require a second‐line therapy. The median treatment‐free survival (TFS) among the 10‐year survivors was 10·5 years (interquartile range 7·4‐12·2). On multivariate analysis independent predictors for TFS were the achievement of complete haematological response and lack of cardiac involvement. Long‐term survivors are increasingly seen in AL amyloidosis and present distinct patient, organ and clonal disease features.