Episode Details

Quick Review #266 - #pathology #oralpathology #doctorgallagher #oralsurgery #oralsurgeon #dentist #dentistry #dental #hemophilia

Hemophilia is a group of inherited bleeding disorders caused by deficiencies in specific clotting factors, leading to impaired blood coagulation and prolonged bleeding.

1 - Hemophilia A (Classic Hemophilia)

• ​ Deficient Factor: Factor VIII (FVIII)
• ​ Genetic Basis: X-linked recessive disorder, primarily affecting males
• ​ Prevalence: The most common form, occurring in approximately 1 in 5,000 male births
• ​ Pathophysiology: Factor VIII is crucial in the intrinsic coagulation pathway, serving as a cofactor for Factor IX in the conversion of Factor X to its active form. Without FVIII, thrombin generation is significantly reduced, resulting in unstable clot formation and prolonged bleeding.
• ​ Clinical Manifestations:
• ​ Spontaneous or excessive bleeding after minor trauma
• ​ Hemarthrosis (bleeding into joints, especially knees, elbows, and ankles)
• ​ Muscle hematomas and deep tissue hemorrhages
• ​ Intracranial hemorrhages, which can be life-threatening
• ​ Prolonged bleeding following surgery or dental procedures

2 - Hemophilia B (Christmas Disease)

• ​ Deficient Factor: Factor IX (FIX)
• ​ Genetic Basis: X-linked recessive disorder, similar to Hemophilia A
• ​ Prevalence: Affects approximately 1 in 25,000 male births
• ​ Pathophysiology: Factor IX is a serine protease involved in activating Factor X within the intrinsic coagulation pathway. Its deficiency results in impaired thrombin production, leading to defective clot formation and prolonged bleeding, similar to Hemophilia A.
• ​ Clinical Manifestations:
• ​ Indistinguishable from Hemophilia A
• ​ Frequent spontaneous joint and muscle hemorrhages
• ​ Prolonged bleeding following trauma or surgical procedures.

3 - Hemophilia C (Factor XI Deficiency)

• ​ Deficient Factor: Factor XI (FXI)
• ​ Genetic Basis: Autosomal recessive disorder, affecting both males and females
• ​ Prevalence: More commonly observed in Ashkenazi Jewish populations
• ​ Pathophysiology: Factor XI is part of the intrinsic coagulation pathway, though it plays a less critical role than FVIII and FIX. Its deficiency leads to variable bleeding patterns, with symptoms generally milder than those seen in Hemophilia A and B.
• ​ Clinical Manifestations:
• ​ Mild to moderate bleeding tendencies
• ​ Prolonged bleeding after surgical procedures, particularly in dental extractions, tonsillectomies, and other invasive procedures

References:

1. ​ Phasura, A. (n.d.). Hemophilia: Damaged blood vessel, Haemophilia coagulation disorder
2. ​ Peyvandi, F., Garagiola, I., & Young, G. (2016). The past and future of hemophilia: Diagnosis, treatments, and its complications. The Lancet, 388(10040), 187–197
3. ​ Franchini, M., & Mannucci, P. M. (2013). Hemophilia A and hemophilia B: The role of coagulation factors and current treatment. Blood Transfusion, 11(4), 487–493
4. ​ ChatGPT.2025

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2.11.2025