Haematologica
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Randomized Controlled Trial Clinical Trial
The effect of two different doses of aprotinin on hemostasis in cardiopulmonary bypass surgery: similar transfusion requirements and blood loss.
Various dosages of aprotinin have proven to be effective in reducing blood loss and allogeneic transfusion requirements in cardiopulmonary bypass surgery, despite the controversy surrounding the precise hemostatic mechanisms of this drug. The aim of our prospective, randomized, double-blind study was to assess differences in blood loss and transfusion requirements and the effect of two dosages of aprotinin on hemostatic activation. ⋯ Our study shows that both dosages of aprotinin are safe and effective in reducing transfusion requirements. Considering the difference in cost of using a low-dose or high-dose schedule, the former should be recommended for patients undergoing cardiopulmonary bypass surgery.
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The prothrombin G20210A mutation and factor V Leiden have been found to be associated with an increased risk of venous thrombosis, but the reported prevalences of the prothrombin gene variant both in the normal population and in patients with deep venous thrombosis (DVT) vary greatly in the literature. Moreover, the influence of oral contraceptives (OC) on thrombotic events in patients with the prothrombin G20210A variant has not been well established. In this study we evaluate both circumstances. ⋯ The prevalence of the prothrombin G20210A mutation in the healthy population in our study is similar to that observed in other southern European countries. The prothrombin G20210A mutation does not by itself seem to be a high thrombotic risk factor. However, when it is present together with other thrombotic risk factors, the predicted risk of thrombotic events increases. The use of OC by women with the prothrombin G20210A variant or FV Leiden, either alone or combined with other thrombotic risk factors, was associated with a significant increase in the risk of venous thrombosis.
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Allogeneic peripheral blood stem cell transplantation with CD34+ cell-selection (CD34+-PBSCT) allows rapid hematologic engraftment with a reduction in graft-versus-host disease (GVHD), although concerns exist regarding the increased risk of tumor relapse associated with T-cell depletion of the graft. Delayed T-cell add-back (TCAB) after such transplants may restore the graft-versus-tumor effect while achieving a reduced early transplant-related mortality due to less GVHD in a group of patients at high risk of early death (i.e., age >= 45 years). ⋯ The results of our pilot study suggest that this protocol produces an acceptable transplant-related morbidity and mortality in patients 45 years and older. However, there may be benefit in infusing CD34+-selected PBSCT with even lower T-cell contents and further delaying the TCAB.
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The North American Immune Tolerance Registry (NAITR) was initiated with the goal of determining, by questionnaire, immune tolerance (ITT) practices in hemophilia treatment centers in Canada and the United States. Sixty-eight centers (40%) responded. Of the 130 registry subjects with hemophilia A who completed ITT, 93 (72%) achieved tolerance. ⋯ The definition of tolerance was reviewed for the 9 subjects who relapsed and was defined by a normal recovery and survival in only 1/9 patients. Among the 11 hemophilia B subjects in the cohort who completed tolerance, 4 had a successful outcome. Four individuals were placed on maintenance regimens of 25-100 units FIX/kg/day and all remained tolerant.
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Immune tolerance induction (ITI) regimens with human factor VIII concentrates are rarely if ever implemented in adult patients with auto-inhibitors, in contrast to alloantibody suppression, which is used primarily in young children with congenital hemophilia. On the basis of some earlier experience with synchronization of plasma exchange therapy of various autoimmune disorders we have developed a new aggressive protocol for the treatment of patients with acquired factor VIII (FVIII) antibody. We have evaluated the outcome of 14 consecutive nonhemophilic FVIII inhibitor patients treated in a single center with our ITI protocol between 1992 and 1999, comparing them to 6 historical control patients, treated with traditional immunosuppression therapy (steroid +/- cyclophosphamide) between 1988 and 1992. ⋯ We conclude that the ITI protocol described here is highly effective for the treatment of acquired hemophilia, induces quick therapeutic responses and favorably influences the underlying autoimmune disorder. We suggest that our ITI protocol is suitable for the eradication of idiopathic and autoimmune-associated FVIII autoantibodies in patients presenting with severe bleeding.