Seminars in hematology
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Seminars in hematology · Jan 2006
ReviewGlanzmann's thrombasthenia treatment: a prospective observational registry on the use of recombinant human activated factor VII and other hemostatic agents.
Glanzmann's thrombasthenia (GT) is a rare congenital bleeding disorder caused by deficiency or dysfunction of platelet surface glycoprotein (GP) IIb/IIIa receptor. Platelet transfusion is the standard treatment for bleeding that remains non-responsive to conservative measures, and for surgical coverage. Platelet transfusions, however, may result in the development of antibodies to GPIIb/IIIa and/or human leukocyte antigen (HLA), rendering further transfusions ineffective. ⋯ Standardized data will be collected using a customized internet-based (www.glanzmann-reg.org) data collection tool. Data collection will begin in 2005 and continue for up to 6 years. Patients of all ages from any country are eligible for inclusion.
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Coagulopathy following massive transfusion is a consequence of post-traumatic and surgical hemorrhage. Bleeding following massive transfusion can occur due to hypothermia, dilutional coagulopathy, platelet dysfunction, fibrinolysis, or hypofibrinogenemia. ⋯ Excessive fibrinolysis and low fibrinogen are also causes of bleeding in these patients. Currently, however, there are several agents that have been reported to be effective for the prophylaxis of hemorrhage in surgical patients, including aprotinin for cardiac surgery, orthopedic surgery, and hepatic transplantation, and the off-label use of recombinant activated factor VII (NovoSeven, Novo Nordisk, Bagsvaerd, Denmark) as rescue therapy for life-threatening hemorrhage.
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Seminars in hematology · Jan 2006
Case ReportsImpact of choice of treatment for bleeding episodes on inhibitor outcome in patients with mild/moderate hemophilia a and inhibitors.
Patients with mild/moderate hemophilia A (MHA) may develop inhibitors to factor VIII (FVIII). In this condition, FVIII clotting activity (FVIII:C) baseline levels may remain stable for some patients, but may be reduced to less than 0.01 U/mL for others. Several risk factors for the development of inhibitors in MHA have been proposed. ⋯ Several treatment options for the control of bleeding in patients with MHA and inhibitors (MHAI) are currently available, and the choice of therapeutic strategy should be given careful consideration; some treatments may produce an anamnestic response, thus delaying the return to FVIII:C baseline levels and adversely affecting the duration of the severe bleeding phenotype. To increase our knowledge of MHAI, a retrospective collection of data is currently being performed among hemophilia centers in France and Belgium. Based on five examples of patients with MHAI collated from preliminary study data, we illustrate the impact on inhibitor outcome of the therapeutic choices used to treat bleeding episodes in these patients.