Summary:
Autologous hematopoietic stem cell transplantation (HSCT) is currently being evaluated as a treatment for autoimmune diseases, including systemic lupus erythematosus (SLE), that are associated with a very severe prognosis. We describe a 27-year-old woman with SLE with a 10-year history of refractory antiphospholipid syndrome (APS). She developed progressive myocardial necrosis despite treatment with corticosteroids, cyclophosphamide (CYC), cyclosporine, and immunopheresis. After conditioning with CYC, fludarabine, and antithymocyte globulin, autologous HSCT using CD34+ selection was performed. After transplantation, the clinical symptoms caused by APS remitted, and the serum anticardiolipin antibody level decreased. Remission has persisted for 21 months after transplantation. Although a longer follow-up is required for the assessment of efficacy, autologous HSCT may cure patients with refractory APS.
This is a preview of subscription content, access via your institution
Relevant articles
Open Access articles citing this article.
-
Haemopoietic stem cell transplantation in Systemic lupus erythematosus: a systematic review
Allergy, Asthma & Clinical Immunology Open Access 18 September 2019
Access options
Subscribe to this journal
Receive 12 print issues and online access
$259.00 per year
only $21.58 per issue
Rent or buy this article
Get just this article for as long as you need it
$39.95
Prices may be subject to local taxes which are calculated during checkout

References
Harris EN . Antiphospholipid antibodies. Br J Haematol 1990; 74: 1–9.
Derksen RHWM, Hasselaar P, Blokzijl L, DE Groot PG . Lack of efficacy of plasma-exchange in removing antiphospholipid antibodies. Lancet 1987; 25: 222.
Neuwelt CM, Daikh DI, Linfoot JA et al. Catastrophic antiphospholipid syndrome. Response to repeated plasmapheresis over three years. Arthritis Rheum 1997; 40: 1534–1539.
Traynor AE, Schroeder J, Rosa RM et al. Treatment of severe systemic lupus erythematosus with high-dose chemotherapy and hematopoietic stem-cell transplantation: a phase I study. Lancet 2000; 356: 701–707.
Burt RK, Traynor AE, Pope R et al. Treatment of autoimmune disease by intense immunosuppressive conditioning and autologous hematopoietic stem cell transplantation. Blood 1998; 92: 3505–3514.
Brunner M, Greinix HT, Redlich K et al. Autologous blood stem cell transplantation in refractory systemic lupus erythematosis with severe pulmonary impairment: a case report. Arthritis Rheum 2002; 46: 1580–1584.
Brown JH, Doherty CC, Allan DC, Morton P . Fatal cardiac failure due to myocardial microthrombi in systemic lupus erythematosus. Br Med J 1988; 296: 1505.
Griffith GC, Vural IL . Acute and subacute disseminated lupus erythematosus: a correlation of clinical and postmortem findings in eighteen cases. Circulation 1951; 3: 492–500.
Euler HH, Marmont AM, Bacigalupo A et al. Early recurrence or persistence of autoimmune diseases after unmanipulated autologous stem cell transplantation. Blood 1996; 88: 3621–3625.
Tyndall A, Gratwohl A . Blood and marrow stem cell transplants in autoimmune disease: a consensus report written on behalf of the European League against Rheumatism (EULAR) and the European Group for Blood and Marrow Transplantation (EBMT). Bone Marrow Transplant 1997; 19: 643–645.
van Bekkum DW . Stem cell transplantation in experimental models of autoimmune disease. J Clin Immunol 2000; 20: 10–16.
Mukai M, Bohgaki T, Kondo M et al. Changes in the T-helper cell 1/T-helper cell 2 balance of peripheral T-helper cells after autologous peripheral blood stem cell transplantation for non-Hodgkin's lymphoma. Ann Hematol 2001; 80: 715–721.
Openshaw H, Nash RA, McSweeney PA . High dose immunosuppression and hematopoietic stem cell transplantation in autoimmune disease: clinical review. Biol Blood Marrow Transplant 2002; 8: 233–248.
Burt RK, Marmont A, Schroeder J et al. Intense immune suppression for systemic lupus – the role of hematopoietic stem cells. J Clin Immunol 2000; 20: 31–37.
Adachi Y, Inaba M, Amoh Y et al. Effect of bone marrow transplantation on antiphospholipid antibody syndrome in murine lupus mice. Immunobiology 1995; 192: 218–230.
Olalla JI, Ortin M, Hermida G et al. Disappearance of lupus anticoagulant after allogeneic bone marrow transplantation. Bone Marrow Transplant 1999; 23: 83–85.
Acknowledgements
We acknowledge Dr S Nishiyama for providing the clinical data and the nursing staff of Hyogo College of Medicine for their assistance in autologous HSCT.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Hashimoto, N., Iwasaki, T., Sekiguchi, M. et al. Autologous hematopoietic stem cell transplantation for refractory antiphospholipid syndrome causing myocardial necrosis. Bone Marrow Transplant 33, 863–866 (2004). https://doi.org/10.1038/sj.bmt.1704432
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1038/sj.bmt.1704432
Keywords
- antiphospholipid syndrome
- hematopoietic stem cell transplantation
- myocardial necrosis
- systemic lupus erythematosus
This article is cited by
-
Haemopoietic stem cell transplantation in Systemic lupus erythematosus: a systematic review
Allergy, Asthma & Clinical Immunology (2019)
-
Successful treatment of a case of catastrophic antiphospholipid syndrome with autologous BMT: case report and review of literature
Bone Marrow Transplantation (2011)
-
Challenges in bringing the bench to bedside in drug development for sle
Nature Reviews Drug Discovery (2004)