VON WILLEBRAND DISEASE

DEFINITION:
VON WILLEBRAND DISEASE

• Von Willebrand disease is a lifelong bleeding disorder

in which your blood doesn't clot properly.

• Blood contains many proteins that help the blood clot

when needed. One of these proteins is called von
Willebrand factor (VWF)
VON WILLEBRAND FACTOR (VWF)
• Von Willebrand factor is a blood glycoprotein involved
in hemostasis, specifically, platelet adhesion.

• This is necessary for normal platelet adhesion, normal

bleeding time, and stabilizing factor VIII.
FACTOR VIII
• is an essential blood-clotting protein, also known as
anti-hemophilic factor.

• The F8 gene provides instructions for making a protein

called coagulation factor VIII.
EPIDEMIOLOGY
• Von Willebrand disease (VWD) occurs with equal frequency
among men and women, affecting up to 1% of the general
population.

• While rare, it is possible for a person to get VWD without a

family history of the disease. This can happen if a
spontaneous mutation occurs.

• However, women are more likely to experience symptoms

of VWD because of the increased bleeding it causes during
their menstrual periods, during pregnancy, and after
childbirth.
ETIOLOGY
• VWD is caused by a defect or deficiency in von
Willebrand Factor (VWF)

• Many people with von Willebrand disease also have

low levels of factor VIII
PATHOPHYSIOLOGY
• VWF is active only in high blood flow condition and
shear stress. Hence the organs with extensive small
vessels such as skin, uterus, and gastrointestinal tract
show deficiency of the factor. The pathophysiology of
different forms of VWD can be given as followed.
PATHOPHYSIOLOGY
CLASSIFICATION OF VON WILLEBRAND DISEASE

• TYPE 1 VWD includes 75% of the cases

▫ type 1 is the mildest form of the disorder
▫ Generally, affected individuals develop mild mucocutaneous
bleeding; in rare cases, affected individuals will develop more
severe symptoms. Nosebleeds and bruising are common
findings for affected children; heavy menstrual bleeding is a
common finding for women of childbearing age.
PATHOPHYSIOLOGY
CLASSIFICATION OF VON WILLEBRAND DISEASE

• TYPE 2 VWD includes 9-30% of the cases

▫ Symptoms are mild to moderate
▫ In these individuals, VWF can be present in the blood in
normal or near normal levels, but doesn’t function properly.
VWD type 2 is further subdivided depending upon the specific:

▫ Subdivided into four types:

 TYPE 2A
 TYPE 2B
 TYPE 2M
 TYPE 2N
TYPE 2 VWD
• TYPE 2A-
• is characterized by reduced VWF that fails to bind to platelets, reducing the ability of
platelets to clump together to form a clot. Affected individuals often have mild to
moderate mucocutaneous bleeding.
• TYPE 2B-
• is characterized by platelets that have an increased ability to clump together, causing
platelets to clump together prematurely in the bloodstream rather than at the site of
blood vessel injury.
• Individuals experience mild to moderate mucocutaneous bleeding and are at a risk of
developing reduced levels of platelets in the blood (thrombocytopenia).
• TYPE 2M-
• is characterized by decreased activity of VWF and its failure to interact with platelets.
This form is generally associated with mild or moderate mucocutaneous bleeding. In
some cases, more severe bleeding episodes will develop.
• TYPE 2N-
• is characterized by the failure of VWF to transport factor VIII to the site of injury and
reduced levels of factor VIII in the blood. Individuals develop excessive bleeding
following surgery.
• This form of VWD can resemble the mild form of classic hemophilia (hemophilia A).
PATHOPHYSIOLOGY
CLASSIFICATION OF VON WILLEBRAND DISEASE

• TYPE 3 VWD accounts for less than 5% of all the cases.

▫ type 3 is the most severe and rarest form of the disorder.
▫ Affected individuals have an almost complete absence of VWF in
their blood.
▫ Affected individuals can experience severe mucocutaneous
bleeding, bleeding into the muscles and joints, joint damage, and
the development of multiple hematomas.
• Acquired VWD
▫ occurs most often in individuals over 40 years with no prior
bleeding history.
▫ not hereditary
VWD TYPE VWF LEVELS % VWD PATIENTS SYMPTOMS
AFFECTED
TYPE 1 Low VWF levels About 75% The most common
form of the disease;
symptoms are
usually mild

TYPE 2 Normal VWF levels About 9-30% Usually cause

but VWF does not moderate symptoms,
work correctly with occasional
severe symptoms

TYPE 3 Little or no VWF Rare, less than 5% Usually causes the

most severe
symptoms
DIAGNOSIS
• The diagnosis is established based on the personal and/or family history of abnormal
bleeding and diagnostic test results.

SCREENING TESTS:

• Complete Blood Count (CBC)

▫ This common test measures the amount of hemoglobin, the size and number of red
blood cells, and the numbers of different types of white blood cells and platelets found
in blood.

• 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).

• 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).

• Fibrinogen Test
▫ This test also helps doctors assess a patient’s ability to form a blood clot. This
test is ordered either along with other blood clotting tests or when a patient has
an abnormal PT or APTT test result, or both.
DIAGNOSIS
• The diagnosis is established based on the personal and/or family
history of abnormal bleeding and diagnostic test results.

DIAGNOSTIC TESTS:

• Factor VIII clotting activity

• Von Willebrand factor antigen
• Ristocetin cofactor or other VWF activity
• Von Willebrand factor multimers
• Platelet aggregation tests
MANAGEMENT AND TREATMENT
The goal of therapy for von Willebrand disease is to increase von
Willebrand factor and factor VIII levels to prevent bleeding during
surgery or arrest bleeding when it occurs.

• Desmopressin is the treatment of choice in patients with Type 1 von

Willebrand's disease, who account for approximately 80% of cases.
• Oral contraceptives
• Clot-stabilizing medications
▫ These anti-fibrinolytic medications such as aminocaproic acid (Amicar) and
tranexamic acid (Cyklokapron, Lysteda) — can help stop bleeding by slowing
the breakdown of blood clots.
• Drugs applied to cuts
▫ A fibrin sealant (Tisseel) placed on a cut helps curtail bleeding. This is applied
like glue using a syringe. There are also over-the-counter products to stop
nosebleeds.
MANAGEMENT AND TREATMENT
• Type 1 VWD
▫ A clinical trial with Desmopressin (DDAVP) is recommended for increasing
endogenous VWF in type 1 disease, although the response is variable.

• Type 2 VWD
▫ Desmopressin (DDAVP) is not always effective. Specifically, in type 2B, DDAVP may
worsen the thrombocytopenia and also cause spontaneous platelet aggregation.
Thus treatment with VWF concentrates is required for maintaining hemostasis in
most type 2 VWD.

• Type 3 VWD
▫ patients are unresponsive to DDAVP and exogenous VWF is the treatment of
choice.
▫ The treatment of choice for patients with vWD type 3 is with or without FVIII or
virus-inactivated, vWF-containing FVIII concentrates that contain a near-normal
complement of high-molecular-weight vWF multimers.
Replacement Therapy

These include infusions of concentrated blood-clotting factors

containing von Willebrand factor and factor VIII. The doctor might
recommend them if Desmopressin isn't an option or has been
ineffective.

o Cryoprecipitate is a plasma-derived blood product for

transfusion that contains fibrinogen (factor I), factor VIII, factor
XIII, von Willebrand factor, and fibronectin.

o Contains approximately 80 to 100 units of von Willebrand factor

per unit (5–10 times more von Willebrand factor and factor VIII
than fresh-frozen plasma), and in the past it was the mainstay
of therapy for von Willebrand disease.
 REPLACEMENT THERAPY FOR VON WILLEBRAND DISEASE
CONDITION THERAPY
Major surgery  Maintain factor VIII level ≥50% for 1
week
 Prolonged treatment in type 3 patients
(>7 days)
Minor surgery  Maintain factor VIII level ≥50% for 1–3
days
 Maintain factor VIII level >20%–30% for
an additional 4–7 days

Dental extraction  Single infusion to achieve factor VIII

level >50%
 Desmopressin prior to procedure for
type I
Spontaneous or post traumatic bleeding  Usually single infusion of 20–40
units/kg
RECOMMENDATION AND MONITORING
Treatment depends on the type of von Willebrand's
disease you have, how much you bleed, and your risk for
heavy bleeding.
You may need to:

• Avoid nonsteroidal anti-inflammatory drugs (NSAIDs)

• Take medicine to prevent heavy bleeding if you have an
injury, are going to have surgery, or are about to give
birth.
• Avoid medicines (called blood thinners)
CASE STUDY
PATIENT HISTORY:

• A 2-year old boy was brought to the emergency

department by his mother for oozing blood from his
mouth following a fall nearly 6 hours ago. His mother
related that he tended to bleed for prolonged periods
from his immunization sites, but there was no history
of bruising or hematomas. The patient was on
antibiotics for a recent ear infection. There was no
known family history of a bleeding disorder.
PHYSICAL EXAMINATION:

• GENERAL: Alert, in no apparent distress, development

appropriate for age

• HEENT (head, eyes, ears, nose, throat): Two small

lacerations on the inside of lower lip, oozing blood

• Remainder of exam within normal limits (notably, no

petechia, bruises, joint swelling)
INITIAL LABORATORY TEST:

ADDITIONAL WORKUP:
Pcot PLAN-FARM
FINDINGS ASSESSMENT RESOLUTION MONITORING

 2-y/o boy  Suspected to  Desmopressin to  Monitoring of

 Oozing blood have Von prevent bleeding medications used
from his mouth Willebrand  Antifibrinolytic (avoid NSAIDs
 Bleed for Disease agents such as and blood
prolonged  Prolonged Oral tranexamic acid thinners)
periods from bleeding  Proper
immunization conducting of
sites screening and
 No history of diagnostic tests
bruising or in determining
hematomas the VWF and its
 Ear infection activities
 No known family
history
 Two lacerations
on the inside of
lower lip
Kurt Adrian R. Parreño

Definition

Von Willebrand disease

- Von Willebrand disease is a lifelong bleeding disorder in which your blood doesn't clot
properly.
- Blood contains many proteins that help the blood clot when needed. One of these
proteins is called von Willebrand factor (VWF) which I’ll be explaining in a moment
- This VWD is a lack of or a defective plasma protein (von Willebrand factor) necessary for
the adhesion of platelets to vascular elements when a blood vessel is damaged.

Von Willebrand factor

- von Willebrand factor is a blood glycoprotein involved in hemostasis, specifically, platelet

adhesion.
- a large multimeric glycoprotein that performs two critical functions in primary hemostasis:
it acts as a bridging molecule at sites of vascular injury under high shear conditions, it
promotes platelet aggregation. This helps form platelet plug during clotting process.
- VWF also helps platelets bind to the inside of injured blood vessels.
- This is necessary for normal platelet adhesion, normal bleeding time, and stabilizing
factor VIII.

Factor VIII

- is an essential blood-clotting protein, also known as anti-hemophilic factor.

- The F8 gene provides instructions for making a protein called coagulation factor VIII.
- is the protein that is deficient or defective in patients with classical hemophilia and Von
Willebrand syndrome
 - Coagulation factors are a group of related proteins that are essential for the formation of
blood clots. After an injury, clots protect the body by sealing off damaged blood vessels
and preventing further blood loss. Is used to prevent or control bleeding in patients with
hemophilia A and even VWD
- is used to prevent or control bleeding in patients with hemophilia A and VWD.

Epidemiology

- Von Willebrand disease (VWD) occurs with equal frequency among men and
women, affecting up to 1% of the general population.
- The most common congenital bleeding disorder in the United States and in the world.
- Unlike hemophilia, von Willebrand disease has an autosomal inheritance pattern,
resulting in an equal frequency of disease in males and females.
- While rare, it is possible for a person to get VWD without a family history of the disease.
This can happen if a spontaneous mutation occurs.
- However, women are more likely to experience symptoms of VWD because of the
increased bleeding it causes during their menstrual periods, during pregnancy, and after
childbirth.

Etiology

- VWD is caused by a defect or deficiency in von Willebrand Factor (VWF)

- The usual cause of von Willebrand disease is an inherited abnormal gene that controls
von Willebrand factor.
- Many people with von Willebrand disease also have low levels of factor VIII
- VWF binds to clotting factor VIII in the circulation and protects it from being broken
down.
- A deficiency of von Willebrand factor reduces the half-life of factor VIII and decreases
plasma factor VIII levels. Therefore, von Willebrand factor plays a dual role in
hemostasis, affecting both platelet function and coagulation.

Pathophysiology

VWF is active only in high blood flow condition and shear stress. Hence the organs with
extensive small vessels such as skin, uterus, and gastrointestinal tract show deficiency of the
factor. The pathophysiology of different forms of VWD can be given as followed.

Classification of Von Willebrand disease

Von Willebrand disease consists of a heterogeneous group of disorders that can be classified
into three major subtypes
Types 1 and 3 are associated with quantitative defects in von Willebrand factor; type 2
mutations refer to functional abnormalities in von Willebrand factor

 Type 1 VWD includes 75% of the cases

- type 1 is the mildest form of the disorder
- Affected individuals may have low levels of VWF in the blood
- In some cases, factor VIII may also be reduced
- Generally, affected individuals develop mild mucocutaneous bleeding; in rare
cases, affected individuals will develop more severe symptoms. Nosebleeds and
bruising are common findings for affected children; heavy menstrual bleeding is a
common finding for women of childbearing age.

 Type 2 VWD includes 9-30% of the cases

- Symptoms are mild to moderate
- In these individuals, VWF can be present in the blood in normal or near normal
levels, but doesn’t function properly. VWD type 2 is further subdivided depending
upon the specific:
- It is a qualitative defect where there is no change in plasma VWF levels but
characterized by a structural and functional defects based on which, it is further
sub-divided into four types
- TYPE 2A- is characterized by reduced VWF that fails to bind to platelets,
reducing the ability of platelets to clump together to form a clot. Affected
individuals often have mild to moderate mucocutaneous bleeding.
- TYPE 2B- is characterized by platelets that have an increased ability to clump
together, causing platelets to clump together prematurely in the bloodstream
rather than at the site of blood vessel injury.
- Individuals experience mild to moderate mucocutaneous bleeding and are at a
risk of developing reduced levels of platelets in the blood (thrombocytopenia).
- Thrombocytopenia condition in which you have a low blood platelet count
- Thrombocytopenia is worsened by stressful situations such as infection,
undergoing surgery, or pregnancy.
- TYPE 2M- is characterized by decreased activity of VWF and its failure to
interact with platelets. This form is generally associated with mild or moderate
mucocutaneous bleeding. In some cases, more severe bleeding episodes will
develop.
- TYPE 2N- is characterized by the failure of VWF to transport factor VIII to the site
of injury and reduced levels of factor VIII in the blood. Individuals develop
excessive bleeding following surgery.
- This form of VWD can resemble the mild form of classic hemophilia (hemophilia
A).

 Type 3 VWD accounts for less than 5% of all the cases.

- Type 3 von Willebrand disease is rare, affecting only about 1 person in
1,000,000.
 - type 3 is the most severe and rarest form of the disorder.
- Affected individuals have an almost complete absence of VWF in their blood.
- Affected individuals can experience severe mucocutaneous bleeding, bleeding
into the muscles and joints, joint damage, and the development of multiple
hematomas.
- For women with the more severe forms of VWD, monitoring for serious, even life-
threatening, reproductive tract bleeding and postpartum hemorrhage is very
important.
-
 Acquired VWD
- occurs most often in individuals over 40 years with no prior bleeding history.
- not hereditary (not transmittable from parent to offspring)

usually in the absence of a personal or family history of bleedings and frequently in

association with monoclonal gammopathies, lymphoproliferative, myeloproliferative
and autoimmune disorders

VWD TYPE VWF LEVELS % VWD PATIENTS SYMPTOMS

AFFECTED
TYPE 1 Low VWF levels About 75% The most common
form of the disease;
symptoms are usually
mild
TYPE 2 Normal VWF levels About 9-30% Usually cause
but VWF does not moderate symptoms,
work correctly with occasional
severe symptoms
TYPE 3 Little or no VWF Rare, less than 5% Usually causes the
most severe
symptoms

Disagnosis

- The diagnosis is established based on the personal and/or family history of abnormal
bleeding and diagnostic test results.
- When a patient has a lifelong history of mucocutaneous bleeding and a family history of
abnormal bleeding, the clinician should suspect von Willebrand disease.
- SCREENING TESTS:
 A combination of blood tests is needed to diagnose the disease. The following
screening tests are done first to show if the blood is clotting properly. These tests
may show if there is a bleeding disorder, but more tests are needed to tell the
 type of bleeding disorder present. Screening tests are often normal in VWD, and
more specific tests are required.
- 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.
- Fibrinogen Test
 This test also helps doctors assess a patient’s ability to form a blood clot. This
test is ordered either along with other blood clotting tests or when a patient has
an abnormal PT or APTT test result, or both.
 The results of this test will be normal among people with VWD. Fibrinogen is
another name for clotting factor I (1).

- 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
- Factor VIII clotting activity
 To measure the amount of factor VIII in the blood
- 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

Management and treatment

The goal of therapy for von Willebrand disease is to increase von Willebrand factor and factor
VIII levels to prevent bleeding during surgery or arrest bleeding when it occurs.

The therapeutic strategies depend on accurate diagnosis and subtyping of VWD.

 Desmopressin is the treatment of choice in patients with Type 1 von Willebrand's

disease, who account for approximately 80% of cases. The pharmacological compound
raises endogenous factor VIII and von Willebrand factor and corrects the prolonged
bleeding time in most patients.
 Oral contraceptives (In addition to preventing pregnancy, these drugs can help control
heavy bleeding during menstrual periods. The estrogen hormones in birth control pills
can boost von Willebrand factor and factor VIII activity.)
 Clot-stabilizing medications (These anti-fibrinolytic medications — such as
aminocaproic acid (Amicar) and tranexamic acid (Cyklokapron, Lysteda) — can help
stop bleeding by slowing the breakdown of blood clots. Doctors often prescribe these
drugs before or after a surgical procedure or tooth extraction)
 Drugs applied to cuts A fibrin sealant (Tisseel) placed on a cut helps curtail bleeding.
This is applied like glue using a syringe. There are also over-the-counter products to
stop nosebleeds.

Desmopressin causes the release of von Willebrand's antigen from the platelets and the cells
that line the blood vessels where it is stored. Von Willebrand's antigen is the protein that carries
factor VIII. This increase in von Willebrand's antigen and factor VIII helps to stop bleeding.

Desmopressin is the drug of choice for mild hemophilia A and von Willebrand disease and (by
unclear mechanisms) for platelet function disorders. In vivo despmopressinDDAVP selectively
and markedly enhances the ability to form procoagulant platelets by enhancing intracellular Na+
and Ca2+ fluxes

Desmopressin stimulates the endothelial cell release of von Willebrand factor and factor VIII. It
is effective for patients with von Willebrand disease who have adequate endogenous stores of
functional von Willebrand factor. This group includes most patients with type 1 disease and
some patients with type 2A disease.

- Type 1 VWD- A clinical trial with Desmopressin (DDAVP) is recommended for

increasing endogenous VWF in type 1 disease, although the response is variable.
 - Type 2 VWD - Desmopressin (DDAVP) is not always effective. Specifically, in type 2B,
DDAVP may worsen the thrombocytopenia and also cause spontaneous platelet
aggregation. (although no cases of thrombosis have been reported). Thus treatment with
VWF concentrates is required for maintaining hemostasis in most type 2 VWD.
- Desmopressin is not usually recommended for treatment of type 2B disease because
the release of additional abnormal von Willebrand factor may exacerbate
thrombocytopenia.
- Type 3 VWD- patients are unresponsive to DDAVP and exogenous VWF is the
treatment of choice. Conversely, desmopressin is not appropriate for patients with type 3
disease, who lack stores of von Willebrand factor
- The treatment of choice for patients with vWD type 3 (as with other vWD types
unresponsive to DDAVP) with or without FVIII or virus-inactivated, vWF-containing FVIII
concentrates that contain a near-normal complement of high-molecular-weight vWF
multimers.

In summary, the diagnosis of VWD may be difficult as the screening tests (APTT and/or
bleeding time) may be normal or only marginally prolonged. Distinguishing the variants is also
challenging and necessary as the management strategies differ.

This desmopressin is available as an injection. It's a synthetic hormone that controls bleeding by
stimulating your body to release more of the von Willebrand factor stored in the lining of your
blood vessels. Many doctors consider desmopressin the first treatment for managing von
Willebrand disease. It can be used before minor surgical procedures to help control bleeding.
You might be given a trial of desmopressin to make sure it's effective for you.

Replacement Therapy

These include infusions of concentrated blood-clotting factors containing von Willebrand factor
and factor VIII. The doctor might recommend them if Desmopressin isn't an option or has been
ineffective.

Cryoprecipitate is a plasma-derived blood product for transfusion that contains fibrinogen

(factor I), factor VIII, factor XIII, von Willebrand factor, and fibronectin.

Cryo is used to prevent or control bleeding in people whose own blood does not clot properly.
This includes patients with serious but rare hereditary conditions such as Hemophilia A (who
lack factor VIII) and von Willebrand disease (who lack von Willebrand factor).

Contains approximately 80 to 100 units of von Willebrand factor per unit (5–10 times more von
Willebrand factor and factor VIII than fresh-frozen plasma), and in the past it was the mainstay
of therapy for von Willebrand disease.

REPLACEMENT THERAPY FOR VON WILLEBRAND DISEASE

CONDITION THERAPY
Major surgery  Maintain factor VIII level ≥50% for 1 week
  Prolonged treatment in type 3 patients (>7
days)
Minor surgery  Maintain factor VIII level ≥50% for 1–3
days
 Maintain factor VIII level >20%–30% for an
additional 4–7 days
Dental extraction  Single infusion to achieve factor VIII level
- Bleeding after dental extractions is very >50%
frequent in patients with von Willebrand  Desmopressin prior to procedure for type I
disease (vWD) and in the past often
necessitated transfusions with factor
VIII/von Willebrand factor concentrates
- since bleeding after dental treatment
may cause severe or even fatal
complications. Moreover, maintenance
of oral hygiene and prevention of
dental diseases is of great significance
to improve the quality of life and avoid
the dangers of surgery
Spontaneous or post traumatic bleeding  Usually single infusion of 20–40 units/kg

However, because cryoprecipitate is not virally inactivated, it should not be used as first-line
treatment. General guidelines for the dosing of replacement therapy in patients with von
Willebrand disease unresponsive to desmopressin are provided in.

(Severe bleeding episodes can be prevented or controlled with intravenous infusions of virally-
inactivated plasma-derived clotting factor concentrates containing both VWF and FVIII.
Depending on the VWD type, mild bleeding episodes usually respond to intravenous or
subcutaneous treatment with desmopressin, a vasopressin analog.)

Recommendation/ Monitoring

Treatment depends on the type of von Willebrand's disease you have, how much you bleed, and
your risk for heavy bleeding.
You may need to:
 Avoid nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin, ibuprofen (Advil or
Motrin, for example), and naproxen (Aleve). Since this can cause vasoconstriction.
 These drugs affect how platelets work and can increase the risk of bleeding.
 Take medicine to prevent heavy bleeding if you have an injury, are going to have surgery, or
are about to give birth.
 Avoid medicines (called blood thinners) which prevent blood clots. Example: warfarin, heparin
Case presentation Pcot Plan-FARM

CASE 325 -- A 2-YEAR-OLD BOY WITH PROLONGED ORAL

BLEEDING

PATIENT HISTORY:

A 2-year old boy was brought to the emergency department by his mother for oozing blood from
his mouth following a fall nearly 6 hours ago. His mother related that he tended to bleed for
prolonged periods from his immunization sites, but there was no history of bruising or
hematomas. The patient was on antibiotics for a recent ear infection. There was no known
family history of a bleeding disorder.

PHYSICAL EXAMINATION:

GENERAL: Alert, in no apparent distress, development appropriate for age

HEENT (head, eyes, ears, nose, throat): Two small lacerations on the inside of lower lip,
oozing blood

Remainder of exam within normal limits (notably, no petechia, bruises, joint swelling)

INITIAL LABORATORY TEST:

ADDITIONAL WORKUP:
o FINDINGS
 2-year old boy
 Oozing blood from his mouth
 Bleed for prolonged periods from immunization sites
 No history of bruising or hematomas
 Ear infection
 no known family history of bleeding disorder
 two lacerations on the inside of lower lip
o ASSESSMENT
 Suspected to have von willebrand disease (note na sinabi ko kanina na pwede
magka VWD kahit di inherited ng parents sa genes depende na rin sa instances
just like sa case study na following a fall that maybe lethal)
 Prolonged oral bleeding
o RESOLUTION
 Desmopressin to prevent the bleeding(useful in treating or preventing bleeding
episodes in patients with von Willebrand disease, haemophilia A and platelet
function defects.)
 Antifibrinolytic agents such as tranexamic acid (can be used orally or
intravenously to treat mild mucocutaneous bleeding.)
 Tranexamic acid is given to stop or reduce heavy bleeding. When you bleed,
your body forms clots to stop the bleeding. (Doctors often prescribe these drugs
before or after a surgical procedure or tooth extraction)
o MONITORING
 Monitoring of medications used (avoid NSAIDs and blood thinners)
 Proper Conducting of screening and diagnostic tests in determining the VWF and
its activities