Autologous stem cell transplantation in immunoglobulin light chain amyloidosis with factor X deficiency

Stefan Cordes, Morie Gertz, Francis K. Buadi, Yi Lin, Martha Lacy, Prashant Kapoor, Shaji K Kumar, Arleigh McCurdy, Angela Dispenzieri, David M Dingli, Suzanne R. Hayman, William Hogan, Rajiv K. Pruthi

Research output: Contribution to journalArticle

3 Scopus citations

Abstract

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 ofR32% (range:R8 toR92%). 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 (rU0.54; PU0.04) and proteinuria (rUS0.54; PU0.04). Our findings support the decision to offer ASCT to factor X-deficient patients as both appropriate and efficacious.

Original languageEnglish (US)
Pages (from-to)101-108
Number of pages8
JournalBlood Coagulation and Fibrinolysis
Volume27
Issue number1
DOIs
StatePublished - 2016

Keywords

  • Amyloidosis
  • Autologous transplantation
  • Factor X deficiency
  • Haematopoietic stem cell transplantation
  • Haemorrhagic disorder

ASJC Scopus subject areas

  • Hematology

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