Article

Acquired hemophilia A

Servizio di Immunoematologia e Trasfusione, Centro Emofilia, Azienda Ospedaliera di Verona, Verona, Italy.
Hematology (Impact Factor: 1.19). 05/2006; 11(2):119-25. DOI: 10.1080/10245330600574185
Source: PubMed

ABSTRACT Acquired hemophilia A is a rare but severe autoimmune bleeding disorder, resulting from the presence of autoantibodies directed against clotting factor VIII. The etiology of the disorder remains obscure, although approximately half of all cases are associated with other underlying conditions. A prompt diagnosis and appropriate management enable effective control of this acquired hemorrhagic disorder: the aims of therapy are to terminate the acute bleeding episode and eliminate or reduce the inhibitor. The recent availability of bypassing agents, first activated prothrombin complex concentrates and then recombinant activated factor VII, has significantly reduced mortality during the acute phase of the disease in patients with high titer inhibitors. On another front, immunosuppressive therapy (corticosteroids and cytotoxic agents, alone or in various combinations) has resulted in long-term inhibitor suppression in up to 70% of the cases. Moreover, new therapeutic strategies (anti-CD20 monoclonal antibody and immune tolerance protocols) are very promising and may further improve the prognosis of acquired hemophilia A.

1 Follower
 · 
353 Views
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Nine unusual bleeding and clotting disorders (or mimickers of such) are described in the format of case presentations, with focus on clinical history, images and diagnostic tests, followed by a discussion of the disease itself and a summarizing clinical teaching point. The disease entities discussed are acquired factor VIII inhibitor, acquired von Willebrand factor inhibitor, haemophilic pseudotumour, Gardner-Diamond syndrome, coumarin-induced skin necrosis, purple toe syndrome, brachiocephalic vein thrombosis with breast enlargement, and leg swelling due to nephrogenic fibrosing dermopathy and lymphoedema. The publication is meant to demonstrate the fascination of clinical coagulation.
    Hamostaseologie 09/2007; 27(3):191-9. · 1.59 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Pharmacists who practice in the critical care setting require a solid background on the causes and consequences of bleeding, as well as the mechanisms of hemostasis. This article provides an overview of these topics. Bleeding and outcomes as a result of surgery and trauma, from medical and pharmacologic causes, and in obstetrics and gynecology are discussed. Patients with brain trauma, those with inherited and acquired bleeding disorders, and patients undergoing therapeutic anticoagulation are addressed, as these are populations at special risk for severe bleeding. Bleeding events as a result of hypothermia, acidosis, and disseminated intravascular coagulation are also discussed, as is the pathophysiology of massive blood loss. Traditional and newer cell-based models of coagulation mechanisms are described and compared. Application of this information in pharmacy practice will help ensure that therapies to manage and arrest blood loss are used appropriately in a wide variety of clinical scenarios.
    Pharmacotherapy 10/2007; 27(9 Pt 2):45S-56S. DOI:10.1592/phco.27.9part2.45S · 2.20 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Acquired hemophilia A is a rare but severe autoimmune bleeding disorder caused by autoantibodies against factor VIII activity and is a potentially life-threatening hemorrhagic disorder. The incidence of acquired hemophilia A has been estimated as 1.48 cases per million per year. The overall rate of death from all causes of acquired hemophilia reaches up to 22%. In this article, the authors describe the case of a 55-year-old man who presented with unusual bleeding after an accident and the fluctuation of his hemostatic parameters during 13 months of follow-up. Initially he had 43 Bethesda unit (BU) inhibitor to factor VIII and <1% of factor VIII activity. The patient was given prednisone and azathioprine therapy (30 and 100 mg/day, respectively) for 4 months, but his hemostatic parameters did not improved during this phase. Then, 2 g cyclophosphamide was injected every 2 days, but no remarkable improvement was observed. Nine months later his inhibitor titers were high. The inhibitor and factor VIII concentrations were assessed 11 times during these 13 months, and the mean level of factor VIII inhibitor was 44 BU (with a minimum of 2 BU and a maximum of 103 BU); the minimum and maximum factor VIII concentrations were <1% and 20%, respectively. The patient experienced hemarthroses, severe epistaxis, hematoma, and gastrointestinal bleeding episodes during this phase. His factor VIII concentration spontaneously and gradually improved and increased to 51.5% 8 months after stopping the treatment with undetectable factor VIII inhibitor.
    Clinical and Applied Thrombosis/Hemostasis 06/2008; 15(5):588-90. DOI:10.1177/1076029608319442 · 1.58 Impact Factor

Preview

Download
3 Downloads
Available from