Acquired von Willebrand Syndrome associated to secondary IgM MGUS emerging after Autologous Stem Cell Transplantation for AL Amyloidosis

Main Article Content

Victor H Jimenez-Zepeda
Hina Qamar
Adrienne Lee
Karen Valentine
Leslie Skeith

Keywords

Amyloidosis, VWS, Bortezomib

Abstract

Acquired von Willebrand syndrome (AVWS) is a rare hemorrhagic disorder that occurs in patients with no prior personal or family history of bleeding. Here, we describe a case of AVWS occurring after autologous stem cell transplantation (ASCT). Interestingly, AVWS developed after bortezomib-based induction and conditioning regimens. Recent evidence suggests that the proximity of the bortezomib therapy to the collection of stem cells with consequent depletion of regulatory T cells after the conditioning regimen could explain some of the unusual autoimmune complications reported in patients receiving bortezomib prior to ASCT. In addition, this patient developed a secondary MGUS post-ASCT, which may have also contributed to the AVWS. To the best of our knowledge, this is the first case of post-ASCT AVWS reported. Prospective data is needed to better elucidate the mechanisms by which these unusual complications occur in patients receiving bortezomib prior to ASCT. 

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References

References:
1. Rosenzweig M, Giralt S, Landau H. Light-chain amyloidosis: SCT, novel agents and beyond. Bone Marrow Transplant. 2013;48:1022-1027.
2. Jimenez-Zepeda VH, Franke N, Reece DE, et al. Autologous stem cell transplant is an effective therapy for carefully selected patients with AL amyloidosis: experience of a single institution. Br J Haematol. 2014;164:722-728.
3. Roy V. Autologous stem cell transplant for Al amyloidosis. Bone Marrow Res. 2012;2012:238961.
4. Comenzo RL. Amyloidosis. Curr Treat Options Oncol. 2006;7:225-236.
5. Dispenzieri A, Buadi F, Kumar SK, et al. Treatment of Immunoglobulin Light Chain Amyloidosis: Mayo Stratification of Myeloma and Risk-Adapted Therapy (mSMART) Consensus Statement. Mayo Clin Proc. 2015;90:1054-1081.
6. Sanchorawala V, Brauneis D, Shelton AC, et al. Induction Therapy with Bortezomib Followed by Bortezomib-High Dose Melphalan and Stem Cell Transplantation for Light Chain Amyloidosis: Results of a Prospective Clinical Trial. Biol Blood Marrow Transplant. 2015;21:1445-1451.
7. Voisin S, Hamidou M, Lefrancois A, Sigaud M, Mahe B, Trossaert M. Acquired von Willebrand syndrome associated with monoclonal gammopathy: a single-center study of 36 patients. Medicine (Baltimore). 2011;90:404-411.
8. Federici AB RJ, Bucciarelli P, Budde U, van Genderen PJ, Mohri H, Meyer D, Rodeghiero F, Sadler JE. Acquired von Willebrand syndrome: data from an international registry. Thromb Haemost. 2000;84:345-349.
9. Franchini M, Lippi G. Acquired von Willebrand syndrome: an update. Am J Hematol. 2007;82:368-375.
10. Lee A, Sinclair G, Valentine K, James P, Poon MC. Acquired von Willebrand syndrome: von Willebrand factor propeptide to von Willebrand factor antigen ratio predicts remission status. Blood. 2014;124:e1-3.
11. Scott JPV, E. A.; Schroeder, T.; Foster, P. A.; Gill, J. C.; Montgomery, R. R. he Von Willebrand factor propolypeptide, Von Willebrand antigen II , distinguishes acquired Von Willebrand syndrome due to decreased synthesis of Von Willebrand factor from AvWS due to increased clearance of vWF. Blood. 1995;86:(Suppl 1).
12. Federici AB, Stabile F, Castaman G, Canciani MT, Mannucci PM. Treatment of acquired von Willebrand syndrome in patients with monoclonal gammopathy of uncertain significance: comparison of three different therapeutic approaches. Blood. 1998;92:2707-2711.
13. Kos CA, Ward JE, Malek K, et al. Association of acquired von Willebrand syndrome with AL amyloidosis. Am J Hematol. 2007;82:363-367.