Treatment patterns and outcome following initial relapse or refractory disease in patients with systemic light chain amyloidosis.

Am J Hematol 2017 Jun 26;92(6):549-554. Epub 2017 Apr 26.

Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA.

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.

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http://dx.doi.org/10.1002/ajh.24723DOI Listing
June 2017
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References

(Supplied by CrossRef)
Molecular mechanisms of amyloidosis
Merlini et al.
N Engl J Med. 2003
Update on treatment of light chain amyloidosis
Mahmood et al.
Haematologica. 2014
Al amyloidosis
Desport et al.
Orphanet J Rare Dis. 2012
Incidence and natural history of primary systemic amyloidosis in Olmsted County, Minnesota, 1950 through 1989
Kyle et al.
Blood. 1992
Primary systemic amyloidosis - comparison of melphalan and prednisone versus placebo
Kyle et al.
Blood. 1978
Role of high-dose melphalan and autologous peripheral blood stem cell transplantation in AL amyloidosis
Sanchorawala et al.
Am J Blood Res. 2012

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