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Hemophilia (Presentation in Emergency Surgery) 

Hemophilia (Presentation in Emergency Surgery)
Chapter:
Hemophilia (Presentation in Emergency Surgery)
Author(s):

J. Matthew Kynes

DOI:
10.1093/med/9780190226459.003.0085
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date: 25 September 2020

Hemophilia is a complex disease of variable severity that affects clotting function and has significant implications in perioperative and emergency care. Hereditary or de novo mutations cause deficiencies in factor VIII or IX production, which may manifest as spontaneous bleeding into joint spaces, muscles, or other sites in severe forms of the disease. Intracranial bleeding is one of the most serious and often fatal complications. In a patient with abnormal bleeding, laboratory results indicative of hemophilia include an increased partial prothromboplastin time (PT), with normal prothrombin time/international normalized ratio (PTT/INR) and normal platelet count. The diagnosis is confirmed with specific factor assays. Advances in prophylaxis with factor replacement have improved outcomes and reduced bleeding episodes in hemophilia. However, patients with hemophilia may present emergently for operation and require factor replacement. In patients that have developed antibodies to factor replacement, clotting factor bypass agents may be required to control bleeding.

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