Antiphospholipid Syndrome in Patients with Systemic Lupus Erythematosus Treated by Autologous Hematopoietic Stem Cell Transplantation.

The association of antiphospholipid antibodies (APLA) with either vascular thrombosis or fetal morbidity is known as the antiphospholipid syndrome (APS). Since systemic lupus erythematosus (SLE) is frequently associated with APS, we analyzed 44 patients with severe and refractory SLE involved in a larger study of autologous hematopoietic stem cell transplantation (HSCT) utilizing cyclophosphamide and antithymocyte globulin (ATG) conditioning to determine the efficacy of this treatment in eliminating APLA and preventing recurrent thrombi. The prevalence of SLE-related APS in our patient population was 63% (45% met Sapporo criteria for definite APS, and 18% had history of APLA positivity with associated clinical APS features). Nineteen patients with APS had lupus anticoagulant (LA) and/or high titers of anticardiolipin antibodies (ACLA) at the entry into study. Ten patients were positive for LA. Fourteen patients showed elevated titers of ACLA, IgG and/or IgM. When followed for up to 30 months after autologous HSCT, 70% of initially LA-positive patients became and remained negative for anticoagulant. 79% demonstrated normalization of ACLA titers during maximum of 28 months follow-up. 64% of patients refractory to chronic anticoagulation regimen were able to discontinue from anticoagulation therapy at average of 6 months post-transplant and remained thrombotic events-free and in complete SLE remission for up to 78 months after HSCT. There was no treatment-related mortality or severe adverse events, including stem cell engrafment-related hematological complications or overhelming bacterial, fungal or viral infection, in any of the 28 SLE-related APS patient. Autologous hematopoietic stem cell transplantation may be performed safely and appears to be effective therapy for eliminating SLE-related APLA and preventing further thrombotic events.