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What is acquired hemophilia?

Learn the facts about this rare, spontaneous, and potentially fatal disease.

What is acquired hemophilia?

Acquired hemophilia occurs when the immune system spontaneously develops an antibody, or inhibitor, against its own coagulation factor, frequently factor VIII (FVIII).1 

What is acquired hemophilia?

Learn the facts about this rare, spontaneous, and potentially fatal disease.

What is acquired hemophilia?

Acquired hemophilia occurs when the immune system spontaneously develops an antibody, or inhibitor, against its own coagulation factor, frequently factor VIII (FVIII).1 

Acquired hemophilia is:

Recognizing and Diagnosing Acquired Hemophilia thumbnail

Acquired Hemophilia Video

See how hematologists and emergency medicine physicians respond when a patient presents with unexplained bleeding.

Diagnosis time matters.

Knowing how to spot the signs and symptoms of acquired hemophilia is vital, so that critical laboratory tests can be performed and a diagnosis can be made.

  • 35% of patients go undiagnosed for more than 7 days3   
  • 70% of patients experience severe or life-threatening bleeding3  

What makes acquired hemophilia difficult to diagnose?

Because it’s so rare, many ER physicians, oncologists, obstetricians, geriatricians, and other health care providers may not be accustomed to seeing acquired hemophilia.

Patients present with a wide variety of underlying conditions3
Chart: Patients present with a wide variety of underlying conditions

Adapted with permission from Knoebl et al.3

Over half of cases are idiopathic.

Adapted with permission from Knoebl et al.3



Over half of cases are idiopathic.
 


Age and sex distribution of patients with acquired hemophilia3
Age and sex distribution of patients with acquired hemophilia

Adapted from Knoebl et al.3

Adapted from Knoebl et al.3

A bimodal age distribution is associated with acquired hemophilia.

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Selected Important Safety Information

WARNING: THROMBOSIS

  • Serious arterial and venous thrombotic events following administration of NovoSeven® RT have been reported
  • Discuss the risks and explain the signs and symptoms of thrombotic and thromboembolic events to patients who will receive NovoSeven® RT
  • Monitor patients for signs or symptoms of activation of the coagulation system and for thrombosis

Warnings and Precautions

  • Serious arterial and venous thrombotic events have been reported in clinical trials and postmarketing surveillance
  • Patients with congenital hemophilia receiving concomitant treatment with aPCCs (activated prothrombin complex concentrates), older patients particularly with acquired hemophilia and receiving other hemostatic agents, and patients with a history of cardiac and vascular disease may have an increased risk of developing thrombotic events

Indications and Usage

NovoSeven® RT (coagulation Factor VIIa, recombinant) is a coagulation factor indicated for:

  • Treatment of bleeding episodes and perioperative management in adults and children with hemophilia A or B with inhibitors, congenital Factor VII (FVII) deficiency, and Glanzmann’s thrombasthenia with refractoriness to platelet transfusions, with or without antibodies to platelets
  • Treatment of bleeding episodes and perioperative management in adults with acquired hemophilia

Important Safety Information

WARNING: THROMBOSIS

  • Serious arterial and venous thrombotic events following administration of NovoSeven® RT have been reported
  • Discuss the risks and explain the signs and symptoms of thrombotic and thromboembolic events to patients who will receive NovoSeven® RT
  • Monitor patients for signs or symptoms of activation of the coagulation system and for thrombosis

Warnings and Precautions

  • Serious arterial and venous thrombotic events have been reported in clinical trials and postmarketing surveillance
  • Patients with congenital hemophilia receiving concomitant treatment with aPCCs (activated prothrombin complex concentrates), older patients particularly with acquired hemophilia and receiving other hemostatic agents, and patients with a history of cardiac and vascular disease may have an increased risk of developing thrombotic events
  • Hypersensitivity reactions, including anaphylaxis, can occur with NovoSeven® RT. Patients with a known hypersensitivity to mouse, hamster, or bovine proteins may be at a higher risk of hypersensitivity reactions. Discontinue infusion and administer appropriate treatment when hypersensitivity reactions occur
  • Factor VII deficient patients should be monitored for prothrombin time (PT) and factor VII coagulant activity (FVII:C). If FVII:C fails to reach the expected level, or PT is not corrected, or bleeding is not controlled after treatment with the recommended doses, antibody formation may be suspected and analysis for antibodies should be performed
  • Laboratory coagulation parameters (PT/INR, aPTT, FVII:C) have shown no direct correlation to achieving hemostasis

Adverse Reactions

  • The most common and serious adverse reactions in clinical trials are thrombotic events. Thrombotic adverse reactions following the administration of NovoSeven® RT in clinical trials occurred in 4% of patients with acquired hemophilia and 0.2% of bleeding episodes in patients with congenital hemophilia

Drug Interactions

  • Thrombosis may occur if NovoSeven® RT is administered concomitantly with Coagulation Factor XIII

Please click here for Prescribing Information

References:

  1. Huth-Kühne A, Baudo F, Collins P, et al. International recommendations on the diagnosis and treatment of patients with acquired hemophilia A. Haematologica. 2009;94(4):566-575.
  2. Bitting RL, Bent S, Li Y, Kohlwes J. The prognosis and treatment of acquired hemophilia: a systematic review and meta-analysis. Blood Coagul Fibrinolysis. 2009;20(7):517-523.
  3. Knoebl P, Marco P, Baudo F, et al; EACH2 Registry Contributors. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). J Thromb Haemost. 2012;10(4):622-631.