Also, among the 59 patients with GT of the international survey, two patients who received a high dose of rFVIIa given as a continuous infusion supplemented by an antifibrinolytic agent, developed pulmonary embolism and a ureteric clot, respectively [16]. Consequently, the use of rFVIIa should be carefully considered particularly in patients

with cardiovascular disease. The use of rFVIIa was approved by the European Medicines Agency in 2004 for the use in patients with GT who became refractory to platelet transfusions or have developed antiplatelet antibodies. Transfusion of platelets has been the most efficient mode of therapy for bleeding episodes and prophylaxis during surgery in patients with GT or BSS. For patients with the milder inherited platelet dysfunctions, platelet transfusions are rarely needed. The major concerns regarding the use of blood components in patients with selleck chemical the severe types of platelet dysfunction are the potential development of allo-immune antibodies against

HLA antigens and/or against the missing platelet glycoproteins (GP), αIIb, β3 or αIIbβ3 in GT and GPI-IX-V in BSS. In one study of GT patients who were exposed to blood components, the frequencies of HLA antibodies were 8/54 (14.8%), for αIIbβ3 antibodies, the frequency was 16/54 (29.6%), and for antibodies against both HLA and αIIbβ3, 5/54 (9.3%) [16]. In C646 in vivo a smaller study of 16 patients with GT, the frequency of HLA-alloantibodies MCE was quite similar. However, for anti-αIIbβ3 antibodies, the frequency was substantially lower than in the larger study (12.5% vs. 39%) [24]. Additional risks of blood component therapy include allergic reactions, transmission of infectious agents, Rh immunization in Rh-negative patients, and on rare occasions – haemolytic transfusion reactions when the donor is type O and recipient is type A [25]. For partial circumvention of the problems related to platelet transfusion, HLA and ABO-matched donors should be sought. If such donors are unavailable, leucocyte-depleted blood components should be used

because it was shown to be effective in reducing the rate of HLA allo-immunization. Use of platelet pheresis from single donors reduces the risk of allo-immunization against αIIbβ3, thereby diminishing the risk of refractoriness to platelet transfusions. Rh negative patients in the child-bearing age, in whom transfusion of Rh positive blood components is unavoidable, should receive anti-D therapy to neutralize the D-antigen. Using family members for donation of platelets is usually convenient, but should not be done if stem-cell transplantation in the affected patient(s) is considered. Blood from family members should be irradiated to prevent transfusion-related graft-versus-host disease. Platelet transfusions after surgery should be continued until wound healing has been achieved [26] and for at least 2 days after severe bleeding episodes have abated.