A weaker association was observed for FV and FVII deficiencies [1

A weaker association was observed for FV and FVII deficiencies [10, 11]. No association between coagulation factor activity level and clinical bleeding severity was observed for FXI Selleckchem EGFR inhibitor deficiency, thus FXI coagulation factor activity does not predict clinical bleeding severity [10, 11]. For FII deficiency, the sample size was too small to report on correlation [11]. The lack of association between coagulation factor activity level and bleeding severity in patients with RBDs may be attributed to the potential role of

other factors in determining bleeding severity, such as platelets and fibrinolytic potential. There is a high degree of variability in the coagulation factor activity levels observed to be necessary to ensure complete absence of bleeding episodes and the levels that correspond with a probability of major spontaneous bleeding in the different rare coagulation deficiencies. The EN-RBD database has proven to be a valuable tool for the extrapolation

see more of information relevant to clinical practice and further validation of bleeding risk assessments. A more detailed evaluation of each single factor deficiency is necessary. A project collecting prospective data from patients with RBDs (PRO-RBDD) has been established with the aim of increasing the knowledge of clinical and therapeutic aspects of these disorders. Establishing a consensus on factor assay methodology is important to ensure that values from different laboratories/centres can be compared and to inform further research into the potential role of global coagulation assays in the accurate prediction of haemorrhagic risk. FP received

speaker fees 上海皓元 from Novo Nordisk and CSL Behring and an unrestricted grant from Novo Nordisk. PJ has received research funding and honoraria from CSL Behring and Octapharma for educational presentations. OS and DM have nothing to disclose. “
“Summary.  Acquired haemophilia (AH) is a rare autoimmune bleeding disorder, which arises as a result of the spontaneous production of autoantibodies against endogenous factor VIII. The breakdown in immune tolerance is thought to be a result of a combination of genetic and environmental factors. Both human leucocyte antigen (HLA) and cytotoxic T lymphocyte antigen 4 (CTLA-4) play an important role in the maintenance of peripheral T-cell tolerance. A higher frequency of HLA class II alleles and single nucleotide polymorphisms of the CTLA-4 gene have been observed in some autoimmune diseases and severe haemophilia A. In 57 patients with AH, significantly higher frequencies of the HLA class II alleles DRB*16 [odds ratio (OR) 10.2] and DQB1*0502 (OR 2.5) have been detected when compared with controls. The CTLA-4 + 49 G allele has also presented with a significantly higher frequency in the same cohort of patients with AH (OR 2.17).

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