Acquired Hemophilia Clinical Presentation

Updated: Jan 10, 2020
  • Author: Sara J Grethlein, MD, FACP; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
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Presentation

History

Unlike patients with hereditary hemophilia, patients with acquired hemophilia do not have a personal or family history of bleeding episodes (see the image below). [2]

Clinical presentation of acquired hemophilia. Clinical presentation of acquired hemophilia.

About half of the cases are associated with other conditions, such as pregnancy, autoimmune disease, and cancer. [2, 12] The other cases are often idiopathic.

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Physical Examination

The clinical picture of acquired hemophilia differs from that of hereditary hemophilia. For instance, intra-articular bleeding episodes, which are typical in congenital factor VIII (FVIII) deficiency complicated by the presence of alloantibodies, are unusual in patients with acquired hemophilia. Instead, hemorrhages into the skin, muscles, or soft tissues and mucous membranes occur in most patients. [2]

Bleeding episodes are more frequent and severe in patients with acquired hemophilia than in patients with congenital hemophilia (see the image below). [14]

Sites of bleeding in patients with acquired hemoph Sites of bleeding in patients with acquired hemophilia (n = 149). This research was originally published in Blood. Collins PW, Hirsch S, Baglin TP, et al. Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors' Organisation. Blood. 2007;109(5):1870-7. © American Society of Hematology.

The etiology underlying the difference in bleeding manifestations between acquired and congenital hemophilia is unknown. [9]

Typical signs of acquired hemophilia A include overt bleeding, epistaxis, gastrointestinal (GI) and urologic bleeding, and retroperitoneal hematomas. [2, 9, 6] Spontaneous bruising and muscle hematomas are most frequent. [5] If untreated, bleeding into the muscles may progress into a compartment syndrome, with compression of the neurovascular bundles. Subglottic hemorrhage may threaten the airway.

Other frequent manifestations of acquired hemophilia include melena, hematuria, and iatrogenic bleeding, particularly after attempts to insert intravenous (IV) lines. Prolonged postpartum bleeding, excessive bleeding after trauma or surgery, and, occasionally, cerebral hemorrhage may also occur. [2]

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Complications

Complications may be divided into hemorrhagic and nonhemorrhagic categories.

Hemorrhagic complications include the following [2, 5, 20] :

  • Epistaxis
  • Gastrointestinal and urologic bleeding
  • Retroperitoneal hematomas
  • Compartment syndrome
  • Prolonged postpartum bleeding
  • Excessive posttraumatic or postoperative bleeding
  • Subglottic or cerebral hemorrhage
  • Thrombocytopenia

Adverse thrombotic events may also occur as a result of hemostatic therapy. Hemarthroses are rare.

Nonhemorrhagic complications include the adverse effects of immunosuppression, such as infection, sepsis, and neutropenia. [20]

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