Hemophilia Definition, Treatment, Complications and Investigations

Hemophilia

Hemophilia is an inherited disorder which affects males and is transmitted by females.

Hemophilia Definition

• It is a X-linked recessive disorder caused by defiiency of factor VIII. This type of hemophilia is called classic hemophilia.
• The another disorder is hemophilia B or Christmas disease which involves males inherited to X­linked recessive trait. It is caused due to defiiency of factor IX.

Hemophilia Clinical Features

• At birth: Newborn with hemophilia is usually healthy,though bleeding from cord and Cephalohematoma may occur.
• In infant: Hemophilia is asymptomatic till 6 to 12 months,when bruising becomes more obvious. Bleeding from the mouth is common.
• During childhood: In severly affected individuals,spontaneous bleeding may occur in joint and muscles,including psosas muscle.
• In adults: Frequency of spontaneous bleeding decreases,but joints may already have been damaged. Intra­cranial hemorrhage is a life­threatening complication. Spontaneous bleeds are common in mildly affcted individuals, but after injury bleeding occurs till appropriate therapy has begun.

Hemophilia Investigations

• APTT is prolonged in hemophilia A and B, but PT is normal.
• Specific factor assay demonstrate reduced factor VIII c in hemophilia A and factor IX in hemophilia B. If the clinical history is strongly suggestive of hemophilia, normal APTT does not exclude the diagnosis.

Hemophilia Treatment

• It is done by fresh blood transfusion when bleeding is in the large mass.
• Factor VIII concentrate is employed in case of hemophilia A and factor IX concentrate is employed in case of hemophilia B.
• A hemophilic will require treatment before any dental procedure. Such patient is managed by IV infusion or cryoprecipitate or factor VIII combined with aminocaproic acid 4 to 6 g QDS.
• For any major surgery or periodontal surgeries or extraction of tooth hemophilic patient should be hospitalized.
  Infusion of factor VIII concentrate is given before surgery and is continued for 48 to 72 hours. Antibiotic should also be given to the patient

Complications Of Hemophilia

• Hepatitis in patients who received multiple transfusion of FFP or cryoprecipitate.
• AIDS in a patient not screened for HIV and treated with FFP or cryoprecipitate.
• Anemia from excessive bleeding.
• Contractures in muscles following intramuscular hematomas.
• Spontaneous intracranial hemorrhage in severe hemophilia.