Another future approach is to alter the immunogenicity or antigen

Another future approach is to alter the immunogenicity or antigenicity of FVIII by developing novel molecules. This might be achieved through PEGylation (covalent attachment of polyethylene glycol polymer chains) or by ‘deimmunization’ in which amino acid residues serving as contact sites with APCs or major histocompatibility class II molecules are eliminated. Patients with negative antibody titres who remain antibody-positive present a new set of challenges. What is the relevance in terms of FVIII pharmacokinetics

and clinical outcome? Does molecular biology have a role in the clinic? Do patients need more FVIII? Do they bleed more often? There has been a published report of a decreased bleeding rate [48] but further investigation is required. ITI is very demanding

for patients and families. Persistent inhibitors are associated with increased morbidity, click here MS-275 nmr mortality and also high cost. Individualized ITI is a goal for the future but additional studies with larger cohorts are required. International trials provide excellent opportunities to investigate FVIII immunology with the aim of achieving better outcomes for patients with inhibitors. Clinical trials such as RES.I.ST and the International ITI study are expected to proof significance and observational studies such as the ObsITI will provide additional valuable information. Knowledge of predictive factors will be useful in planning more successful ITI. Assessing the FVIII-specific B-cell-mediated immune response appears to be a feasible approach to identifying

newer strategies for the 上海皓元 prevention and elimination of inhibitors in patients with severe haemophilia A. G Di Minno, E Santagostino, K Pratt and C Königs received an honorarium from Grifols S.A. for participating in the symposium and production of the article. The authors thank Content Ed Net for providing editorial assistance in the preparation of the article, with funding by Grifols S.A. “
“Summary.  If continuous prophylaxis is not feasible due to expense or lack of venous access, we must aggressively treat major haemarthroses (including arthrocentesis) to prevent progression to synovitis, recurrent joint bleeds, and ultimately end-stage osteoarthritis (haemophilic arthropathy). For the treatment of chronic haemophilic synovitis, radiosynovectomy should always be indicated as the first procedure. If, after three procedures with 6-month interval, radiosynovectomy fails, an arthroscopic synovectomy must be indicated. Between the second and fourth decades, many haemophilic patients develop joint destruction (arthropathy). At this stage possible treatments include alignment osteotomy, arthroscopic joint debridement, arthrodesis (joint fusion) and total joint arthroplasty.

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