International recommendations on the diagnosis and treatment of patients with acquired hemophilia A Export

@article{citeulike:4311922, abstract = {Acquired hemophilia A (AHA) is a rare bleeding disorder characterized by autoantibodies directed against circulating coagulation factor (F) VIII. Typically, patients with no prior history of a bleeding disorder present with spontaneous bleeding and an isolated prolonged aPTT. AHA may, however, present without any bleeding symptoms, therefore an isolated prolonged aPTT should always be investigated further irrespective of the clinical findings. Control of acute bleeding is the first priority, and we recommend first-line therapy with bypassing agents such as recombinant activated FVII or activated prothrombin complex concentrate. Once the diagnosis has been achieved, immediate autoantibody eradication to reduce subsequent bleeding risk should be performed. We recommend initial treatment with corticosteroids or combination therapy with corticosteroids and cyclophosphamide and suggest second-line therapy with rituximab if first-line therapy fails or is contraindicated. In contrast to congenital hemophilia, no comparative studies exist to support treatment recommendations for patients with AHA, therefore treatment guidance must rely on the expertise and clinical experience of specialists in the field. The aim of this document is to provide a set of international practice guidelines based on our collective clinical experience in treating patients with AHA and contribute to improved care for this patient group. 10.3324/haematol.2008.001743}, author = {Huth-Kuhne, Angela and Baudo, Francesco and Collins, Peter and Ingerslev, Jorgen and Kessler, Craig M. and Levesque, Herve and Castellano, Maria E. and Shima, Midori and St-Louis, Jean}, citeulike-article-id = {4311922}, citeulike-linkout-0 = {http://dx.doi.org/10.3324/haematol.2008.001743}, citeulike-linkout-1 = {http://www.haematologica.org/cgi/content/abstract/94/4/566}, citeulike-linkout-2 = {http://view.ncbi.nlm.nih.gov/pubmed/19336751}, citeulike-linkout-3 = {http://www.hubmed.org/display.cgi?uids=19336751}, day = {1}, doi = {10.3324/haematol.2008.001743}, journal = {Haematologica}, keywords = {aquired, diagnosis, guidelines, inhibitors, international}, month = {April}, number = {4}, pages = {566--575}, posted-at = {2009-04-15 09:46:02}, priority = {2}, title = {International recommendations on the diagnosis and treatment of patients with acquired hemophilia A}, url = {http://dx.doi.org/10.3324/haematol.2008.001743}, volume = {94}, year = {2009} }

TY - JOUR ID - citeulike:4311922 L3 - citeulike-article-id:4311922 N2 - Acquired hemophilia A (AHA) is a rare bleeding disorder characterized by autoantibodies directed against circulating coagulation factor (F) VIII. Typically, patients with no prior history of a bleeding disorder present with spontaneous bleeding and an isolated prolonged aPTT. AHA may, however, present without any bleeding symptoms, therefore an isolated prolonged aPTT should always be investigated further irrespective of the clinical findings. Control of acute bleeding is the first priority, and we recommend first-line therapy with bypassing agents such as recombinant activated FVII or activated prothrombin complex concentrate. Once the diagnosis has been achieved, immediate autoantibody eradication to reduce subsequent bleeding risk should be performed. We recommend initial treatment with corticosteroids or combination therapy with corticosteroids and cyclophosphamide and suggest second-line therapy with rituximab if first-line therapy fails or is contraindicated. In contrast to congenital hemophilia, no comparative studies exist to support treatment recommendations for patients with AHA, therefore treatment guidance must rely on the expertise and clinical experience of specialists in the field. The aim of this document is to provide a set of international practice guidelines based on our collective clinical experience in treating patients with AHA and contribute to improved care for this patient group. 10.3324/haematol.2008.001743 IS - 4 JF - Haematologica EP - 575 TI - International recommendations on the diagnosis and treatment of patients with acquired hemophilia A VL - 94 SP - 566 KW - aquired KW - diagnosis KW - guidelines KW - inhibitors KW - international AU - Huth-Kuhne, Angela AU - Baudo, Francesco AU - Collins, Peter AU - Ingerslev, Jorgen AU - Kessler, Craig AU - Levesque, Herve AU - Castellano, Maria AU - Shima, Midori AU - St-Louis, Jean PY - 2009/04/01/ UR - http://dx.doi.org/10.3324/haematol.2008.001743 ER -