Hemophilia A is the most common form of hemophilia, caused by reduced levels or activity of coagulation factor VIII. This leads to prolonged and unstable clot formation when bleeding occurs. Symptoms can range from bleeding after circumcision or venipuncture in infants to spontaneous bleeding in joints in older patients. Diagnosis involves tests that show prolonged activated partial thromboplastin time and low factor VIII levels. Treatment replaces the missing clotting factor, with severity and location of bleeding determining dosage. Preventative treatment and education on self-administered factor replacement can help manage symptoms.

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The Hemophilia A is The most common form of hemophilia
and, after von Willebrand disease is the second genetic
disorder Associated with bleeding serious.
It is a disease hereditary Caused by a reduction in the amount
or activity of coagulation factor VIII .
This protein acts as a cofactor for factor IX in the activation of
factor X in the coagulation cascade .
A failure at this point in the coagulation cascade results in the
formation of fibrin deficient, que causes coagulation much
more Prolonged and more stable clot.
Approximately 1 in 5,000 evils Affected.
Hemophilia A

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The disease is a recessive Caused by allele of the gene located at F8 X
chromosome ( Xq28 ), so That it develops in men and in women
homozygous .
However, a mild form Described of the disease in females
heterozygous , probably due to a Lyonization unfavorable (normal X
chromosome inactivation in Most cells). Approximately 30% of
patients have no family history, so presumably Their disease is due
to mutations new.
Women with a defective factor VIII gene are carriers of this trait.
50% of the male offspring of female carriers Have the disease and
50% of the female offspring are carriers.
Also, all the daughters of a hemophiliac male carrying the trait.
Hemophilia A Etiology

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Those Affected by May hemophilia have bleeding in any area, but
The most common sites are the joints ( knees , ankles , elbows ),
muscles and digestive system .
The hemarthrosis are so characteristic of spontaneous hemophilia
That are almost the primary diagnosis of the disease.
Patients with mild hemophilia have bleeding only in response to
major trauma or surgery.
And there are Those with severe hemophilia spontaneous bleeding.
The first signs of hemophilia Already May Occur With the delivery
using forceps or vacuum extraction vaginal deliveries, resulting in
the head bruises or hematomas and intracranial hemorrhage events.
Prolonged bleeding from a circumcision or venipuncture is another
common early signs of hemophilia.
Hemophilia A Clinical

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You can believe or suspect the diagnosis to patients with hemophilia
secondary to trauma or spontaneous bleeding, Especially If They
Appear early in life or at birth.
The detailed evaluation of the bleeding history of the patient and his
family will be an important guide for the correct diagnosis of
hemophilia aid. Below laboratory participation is essential.
In patients with coagulopathies congenital abnormalities are evident
on tests measuring clotting globally.
Specifically in the case of hemophilia A is elongated the cephalin .
Confirmation of the type of hemophilia is Obtained When an absence
or significant decrease detected coagulation factor VIII .
Hemophilia A Diagnosis

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Hemophilia A Laboratory

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Tests of blood clotting Many take place if the person tested
is the first in the family with a bleeding disorder Once the
defect has-been Identified, other family members will need
less testing to diagnose. Tests include:
 Part Time thromboplastin time Prolonged.
 Prothrombin time standard.
 Normal bleeding time.
 Normal levels of fibrinogen .
 Low activity of factor VIII serum .
Hemophilia A Laboratory

9. The treatment Involves Replacing the standard clotting factor missing. The
amount of factor VIII concentrates needed depends on the severity and location of
the bleeding, as the size of the patient. Mild hemophilia May be TREATED WITH
desmopressin (DDAVP), que Helps the body release That factor VIII is stored in
the inner lining of blood vessels .
To Prevent a bleeding crisis and Their People with hemophilia can be-taught
families how to administer factor VIII concentrates in Their homes, at the first
signs of bleeding. People with severe forms of the disease May need Regular
preventive treatment.
Depending on severity of the disease, May be Administered factor VIII
concentrate or desmopressin (DDAVP) before performing an extraction dental
and surgery in order to Prevent bleeding. Necessary It is vaccination against
hepatitis B , as there is an Increased risk of exposure to hepatitis B virus , due to
frequent infusions of blood products. Patients who Develop an inhibitor to factor
VIII May require treatment With Other clotting factors: such as clotting factor VIIa
, que can help even in the absence of clotting factor VIII.
Hemophilia A Treatment

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Hemophilia A Treatment

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BY :-
1. Mustafa Ahmed Sulaiman
2. Hussein Salim Hamad
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