HEMOPHILIA

NAME : YOGA ANGGA TRINANDA

CLASS : 3B
 Definition

Hemophilia is a disease that causes bleeding disorders due

to lack of clotting factors. As a result, bleeding lasts
longer when the body is injured. Under normal
circumstances, the protein that becomes the blood clotting
factor forms a retaining web around the platelets (blood
cells) that can freeze the blood and eventually stop the
bleeding. In people with hemophilia, the lack of protein
that became the clotting factor of the blood resulted in
prolonged bleeding.
In addition to the above hereditary factors, there is also the
possibility for a person to have hemophilia A or B through a
spontaneous gene mutation. The genes that cause hemophilia
C can be transmitted to children by one parent. Hemophilia C
can occur in boys and girls. As a result of the gene disorder it
occurs:
Hemophilia A. The most common type, hemophilia A is
caused by a lack of clotting factor 8 (VIII).
Hemophilia B. Due to lack of clotting factor 9 (IX).
Hemophilia C. This type is caused by a lack of freezing
factor 11 (XI), and the symptoms are often mild among the
other types.
 Cause

The process of blood coagulation requires

elements in the blood, such as platelets and blood
plasma proteins. In the case of hemophilia, there
are gene mutations that cause the body to lack
certain clotting factors in the blood. The cause of
hemophilia A is a gene mutation occurring in
clotting factor VI While hemophilia B is caused by
mutations that occur in the clotting factor IX (9) in
the blood. Mutation of genes in hemophilia A and
B occurs on the X chromosome and can be derived
from the father, mother, or both parents.
 Most women can become carriers of this abnormal gene
and lower it to their children, without themselves
experiencing the symptoms of hemophilia. While men
with abnormal genes are likely to suffer from hemophilia.
On the other hand, these gene mutations may also occur
spontaneously in hemophiliacs who have no family
history of hemophilia
 Symptoms

One of the most common signs of hemophilia is

bruising on the skin. Bruise is a bluish skin color that
indicates that there is bleeding under the skin. For
example, occur in muscles or joints. In normal
people also sometimes experience blueness in some
parts of the body like that, but will disappear within
a few days. But if the bruise covers a large area of the
body, or accompanied by red swelling around it, then
immediately see a doctor. It could be a symptom of
hemophilia.
In men who have hemophilia, often the sign is obtained
when they are circumcised. Bleeding that happens due to
circumcision will be long stop. If that happens, then
parents should be wary of the possibility of hemophilia.
 Complications

the result of bleeding or blood transfusions. Complications due

to bleeding are anemia, ambulasis or joint deformity, muscle
atrophy or neuritis.
Damage to joints and musclescomplications of hemophilia
disease
Haematuria, when a blood clot occurs in the urethra, it can
cause sharp pain.
Bleeding digestive system, abnormalities that can arise in the
form of blood in the stool and vomiting. Chronic blood loss
due to this can cause anemia in the patient.
Intracranial haemorrhage
compartment syndrome
 Supporting investigation

a) Skin testing for blood coagulation.

b) Liver biopsy: used to obtain tissue for pathology and

culture checks.

c) Liver function test Used to detect liver disease

 Medical Management

Periodic transfusion of fresh frozen plasma (PBS)

Giving factor VIII and IX concentrates on clients
who experience bleeding active or as a preventive
measure prior to tooth extraction and surgery
Avoid giving aspirin or injections IM
Cleaning the mouth as a preventive measure
Splints and orthopedic devices for clients who
experience muscle and joint bleeding.
THANKS FOR YOUR
ATTENTION !