BLEEDING

DISORDERS 
Done By: Dr. Suzy Qasem Sulaiman 
Supervised by:  Hussien Hilal
Hemostasis and Coagulation
Cascade 
•After the end of clot formation when the
injury is fully healed, the lysis of the fibrin clot
begins with the use of plasmin protein in the
blood 
So, A Bleeding  Disorder
is a problem in any step
of the hemostasis and
Coagulation Cascade 
 • unexplained and easy bruising
• heavy menstrual bleeding
• frequent nosebleeds
What are the
signs and • excessive bleeding from small cuts
symptoms of a or an injury
bleeding • bleeding into joints
disorder? 
Von Willebrand's
Disease
Introduction: 
• Von Willebrand disease (VWD) is a blood disorder in which the blood
does not clot properly. Blood contains many proteins that help the blood
clot when needed. One of these proteins is called von Willebrand factor
(VWF). People with VWD either have a low level of VWF in their
blood or the VWF protein doesn’t work the way it should.
 Epidemiology of the disease 

• Von Willebrand disease is an inherited condition that results when the blood lacks functioning von
Willebrand factor, a protein that helps the blood to clot and also carries another clotting protein, factor
VIII. It is usually milder than hemophilia and can affect both males and females. Women are especially
affected by von WIllebrand disease during menses. Von Willebrand disease is classified into three
different types (Types 1, 2, and 3), based on the levels of von Willebrand factor and factor VIII activity
in the blood. Type 1 is the mildest and most common form; Type 3 is the most severe and least common
form.
What's wrong in Von Willebrand's Disease ? 
What are the Sign&
symptoms of von
Willebrand disease?
• Nosebleeds: These are nosebleeds that last longer
than 10 minutes and happen five or more times a
year.
• Bleeding from a cut or other injury that lasts
longer than 10 minutes.
• Bruises: People with von Willebrand disease
bruise easily. Their bruises are raised, meaning
the bruises look like they’re swollen, and their
bruises are larger than a quarter.
• Iron-deficiency anemia: All anemia happens
when you don’t have enough red blood cells. In
iron-deficiency anemia, your body doesn’t have
enough iron to make hemoglobin. Hemoglobin is
the substance in your red blood cells that helps
them carry oxygen.
• Post-surgery bleeding: People with von
Willebrand disease may have heavy bleeding after
any surgery, including dental surgery.
• Heavy periods (menstrual bleeding): This is
bleeding that’s so heavy you need to change your
pad or tampon every hour, or have bleeding that
lasts longer than seven days.
• Heavy bleeding after childbirth or miscarriage.
• Blood in (stool): 
• Blood in pee (hematuria): Talk to your
healthcare provider if you notice blood when
you pee, particularly if you have an urgent need to
pee and there’s blood in your pee.
Diagnosis 
• To find out if a person has von Willebrand disease (VWD), the doctor will ask questions about
personal and family histories of bleeding. The doctor also will check for unusual bruising or other
signs of recent bleeding and order some blood tests that will measure how the blood clots. The tests
will provide information about the amount of clotting proteins present in the blood and if the
clotting proteins are working properly
Screening Tests

• Complete Blood Count (CBC)

• This common test measures the amount of hemoglobin (the red pigment inside red blood cells that
carries oxygen), the size and number of red blood cells, and the numbers of different types of white
blood cells and platelets found in blood. The CBC is normal among people with VWD. However, if
a person with VWD has unusually heavy bleeding or bleeds for a long time, the hemoglobin and the
red blood cell count can be low
• Activated Partial Thromboplastin Time (APTT) Test
• This test measures how long it takes for blood to clot. It measures the clotting ability of factors VIII
(8), IX (9), XI (11), and XII (12). If any of these clotting factors is too low, it will take longer than
normal for the blood to clot. The results of this test will show a longer clotting time among some
people with VWD. However, the results of this test will be normal among people with mild VWD.
• Prothrombin Time (PT) Test
• This test also measures the time it takes for blood to clot. It measures primarily the clotting ability
of factors I (1), II (2), V (5), VII (7), and X (10). If the level of any one of these factors is too low, it
will take longer than normal for the blood to clot. The results of this test will be normal among most
people with VWD.
Diagnostic Tests
• Specific tests are required to diagnose which bleeding disorder is there. Often these tests need to be repeated several
times before an accurate diagnosis can be made. This is because the levels of clotting factors in the blood vary over
time as a result of changes the body might be reacting to―such as stress, pregnancy, and infections―that can affect
the test results.
• The blood tests that a doctor can order to diagnose VWD (or another platelet disorder) include:

• Von Willebrand factor antigen―To measure the amount of VWF in the blood
• Ristocetin cofactor or other VWF activity―To measure how well the VWF works
• Von Willebrand factor multimers―To measure the makeup or structure of the VWF
• Platelet aggregation tests―To measure how well the platelets are working
• Factor VIII clotting activity―To measure the amount of factor VIII in the blood
Treatment 
• Desmopressin Acetate Injection
• This medicine (DDAVP®) is injected into a vein to treat people with milder forms of VWD (mainly Type 1). It
works by making the body release more VWF into the blood. It also helps increase the level of factor VIII in the
blood.
• Desmopressin Acetate Nasal Spray
• This high-strength nasal spray (Stimate ®) is used to treat people with milder forms of VWD (mainly Type 1). It
works by making the body release more VWF into the blood.
• Factor Replacement Therapy
• Recombinant VWF (such as Vonvendi ®) and medicines rich in VWF and factor VIII (for example, Humate P ®,
Wilate®, Alphanate®, or Koate DVI®) are used to treat people with more severe forms of VWD or people with
milder forms of VWD who do not respond well to the nasal spray. These medicines are injected into a vein in the
arm to replace the missing factor in the blood.
• Antifibrinolytic Drugs
• These drugs (for example, Amicar ®, Lysteda®) are either injected or taken orally to help slow or
prevent the breakdown of blood clots.
• Birth Control Pills
• Birth control pills can increase the levels of VWF and factor VIII in the blood and reduce menstrual
blood loss. A doctor can prescribe these pills for women who have heavy menstrual bleeding.
References: 

• https://www.sciencedirect.com/science/article/abs/pii/S2468785
522000337
• https://www.cdc.gov/ncbddd/vwd/diagnosis.html

• https://www.cdc.gov/ncbddd/vwd/facts.html#:~:text=Von%20W
illebrand%20disease%20(VWD)%20is,von%20Willebrand%20
factor%20(VWF
).
• https://www.hematology.org/education/patients/bleeding-disord
ers#:~:text=Bleeding%20disorders%20such%20as%20hemophi
lia,the%20blood's%20natural%20clotting%20factors
.