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.

Indications and Usage

NovoSeven® RT (Coagulation Factor VIIa [Recombinant]) is a coagulation factor indicated for:

  • Treatment of bleeding episodes and peri-operative 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 peri-operative 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.
  • Exercise caution when administering NovoSeven® RT to patients with an increased risk of thromboembolic complications, such as those with disseminated intravascular coagulation (DIC), advanced atherosclerotic disease, crush injury, septicemia, uncontrolled post-partum hemorrhage, history of coronary heart disease, liver disease, post-operative immobilization, in elderly patients, in neonates, or in patients receiving concomitant treatment with aPCCs/PCCs (activated or nonactivated prothrombin complex concentrates).
  • Hypersensitivity reactions, including anaphylaxis, have been reported with NovoSeven® RT. Administer only if clearly needed in patients with known hypersensitivity to NovoSeven® RT, any of its components, or mouse, hamster, or bovine proteins. Should symptoms occur, discontinue NovoSeven® RT and administer appropriate treatment.
  • 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.