Autologous stem cell transplantation in immunoglobulin light chain amyloidosis with factor X deficiencyCordes, Stefana; Gertz, Morie A.a,b; Buadi, Francis K.a,b; Lin, Yia,b; Lacy, Martha Q.a,b; Kapoor, Prashanta,b; Kumar, Shaji K.a,b; McCurdy, Arleigha,b; Dispenzieri, Angelaa,b; Dingli, Davida,b; Hayman, Suzanne R.a,b; Hogan, William J.a,b; Pruthi, Rajiv K.a,bBlood Coagulation & Fibrinolysis: January 2016 - Volume 27 - Issue 1 - p 101–108 doi: 10.1097/MBC.0000000000000367 ORIGINAL ARTICLES Buy SDC Abstract Author InformationAuthors Article MetricsMetrics Acquired factor X deficiency and associated haemorrhage can be consequences of immunoglobulin light chain amyloidosis. There are limited data on the safety and efficacy of autologous stem cell transplant (ASCT) on factor X deficiency. We retrospectively reviewed immunoglobulin light chain amyloidosis patients with factor X levels below 50%, not on chronic anticoagulation who underwent ASCT at the Mayo Clinic, Rochester, Minnesota, USA, between April 1995 and December 2011. Twenty-seven of 358 patients (7.5%) met study criteria. Median pre-ASCT factor X was 36% (range: 2–49%). The most frequent and severe bleeding complications occurred in patients with factor X levels below 10%. Peri-procedural prophylaxis included activated recombinant factor VII, fresh frozen plasma and platelet transfusions. Steady-state post-ASCT factor X levels were determined in 12 patients. Post-ASCT factor X levels increased in 100% of patients, with median factor X improvement of +32% (range: +8 to +92%). About 46.2% of patients were no longer factor X deficient after ASCT. The degree of improvement in factor X levels was correlated with an improvement in markers of renal involvement by amyloid. Improvement in factor X correlated with an improvement in the degree of total serum protein (ρ = 0.54; P = 0.04) and proteinuria (ρ = −0.54; P = 0.04). Our findings support the decision to offer ASCT to factor X-deficient patients as both appropriate and efficacious. aDepartment of Internal Medicine bDivision of Hematology, Mayo Clinic, Rochester, Minnesota, USA Correspondence to Morie A. Gertz, Division of Hematology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA Fax: +1 507 266 4972; e-mail: Gertz.Morie@Mayo.edu Received 4 September, 2014 Revised 17 May, 2015 Accepted 28 May, 2015 Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Website (www.bloodcoagulation.com). Copyright © 2016 YEAR Wolters Kluwer Health, Inc. All rights reserved.