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1 Blood 1 (Bleeding Disorders)

Bleeding disorders are conditions characterized by the inability to form proper blood clots, leading to excessive bleeding after injuries or surgeries. They can be caused by various factors including vascular disorders, platelet disorders, and coagulation factor deficiencies such as hemophilia and von Willebrand's disease. Treatment typically involves clotting factor replacement, plasma transfusions, and medications to manage bleeding episodes.

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9 views

1 Blood 1 (Bleeding Disorders)

Bleeding disorders are conditions characterized by the inability to form proper blood clots, leading to excessive bleeding after injuries or surgeries. They can be caused by various factors including vascular disorders, platelet disorders, and coagulation factor deficiencies such as hemophilia and von Willebrand's disease. Treatment typically involves clotting factor replacement, plasma transfusions, and medications to manage bleeding episodes.

Uploaded by

nnm65771
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Download as PPTX, PDF, TXT or read online on Scribd
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Bleeding Disorders

What is a Bleeding
Disorder?
 Bleeding disorders:-
 Are a group of disorders that share the
inability to form a proper blood clot.
 They are characterized by extended
bleeding after injury,
surgery, trauma or menstruation.
Hemostasis

 Hemostasis, the arrest of bleeding from an injured blood


vessel, requires the combined activity of :-
 Vascular factors.
 Platelets.
 Plasma coagulation factors.
 Regulatory mechanisms counterbalance the tendency of
clots to form.
 Hemostatic abnormalities can lead to excessive bleeding or
thrombosis.
Mechanisms of Hemostasis
 Vascular disorders:-
 Scurvy
 Purpura
 Hereditary hemorrhagic telangiectasia
 Cushing syndrome
 Fibrinolytic defects:-
 Streptokinase therapy
 Disseminated intravascular coagulation
Platelet disorders

 Quantitative disorder (thrombocytopenia):-


 Immune-mediated
 Idiopathic
 Drug-induced
 Collagen vascular disease
 Leukaemia
 Qualitative:-
 lanzmann thrombasthenia
 Liver disease
 Alcoholism
Coagulation factor deficiencies

 Congenital:-
 Hemophilia A and B
 Von Willebrand’s disease
 Other factor deficiencies (rare)
 Acquired
 Liver disease
 Vitamin K deficiency, warfarin use
 Disseminated intravascular coagulation
Coagulation factor
deficiencies
Hemophilia
 Hemophilia A : is due to a deficiency of clotting factor VIII
or antihemophilic factor or Hemophilia B is due to deficiency
of clotting factor IX (hemophilia B).
 Inherited X-link disorder.
 Prevelance 1 in 5000 in male population.
HEMOPHILIA A : X-LINKED
Clinical features of H.A

Atypical profuse bleeding at circumcision.


Joints and soft tissue bleeds and excessive
bleeding when they start to be active.
 Prolonged bleeding after teeth extraction.
 Recurrent painful hemarthrosis.
 Muscle haemoatomas.
 Spontaneous haematouria.
 GIT hemorrhage.
 Spontaneous intracranial hemorrhage (rare).
Clinical features cont,

 The clinical severity depend on the level


of factor VIII:C = severity of condition
 Severe = factor level < 1%
 Moderate = factor level 1 – 5%
 Mild = factor level > 5 %
Laboratory finding and investigations

Coagulation testing:-
 Prolonged activated partial thromboplastin
time (APTT).
 Normal prothrombin time (PT).
 Normal bleeding time (BT).
 Factor assay (reduced level of factor VIII).
HEMOPHILIA B

 Also known as Christmas disease.


 Caused by a deficiency of factor IX.
 The inheritance and clinical features are identical
to hemophilia A.
 Only can be distinguished by specific coagulation
factor assays.
 The incidence is only about 1 in 30 000 males.
 Hemophilia B is treated with factor IX
concentrates.
VON WILLBRAND’S DISEASE

 Hereditary coagulation abnormality caused by either:-


 Reduced level of vWF.
 Abnormality in vWF.
 Vwf is a protein plays two role in action:-
 It promote adhesion of platelets to the
endothelium.
It is a carrier molecule for factor VIII, protecting it
from premature.
Destruction.
 So in Vwd :-
 Defective platelet function
 Factor VIII:C deficiency
CLASSIFICATION OF VWD:

 3 TYPES:-
 TYPE I:-
 70%-80%.
 Partial loss in quantity.
 TYPE II:-
 Poor in quality.
 TYPEIII:-
 severe loss in quantity.
 The severity of symptoms are variable with types :
 Type 1, 2 usually mild symptoms.
 Type 3 severe symptoms.
Laboratory Investigations:

The bleeding time is prolonged.


 APTT is prolonged.
 Factor VIII is low.
 VWF is usually low (type 1,2).
 Platelets count is normal.
OTHER COAGULATIONS FACTORS

Factor XI deficiency:-
• Rare
• Seen mainly in Ashkenazi Jews
• Caused bleeding only after trauma
• Treated by factor XI
 Factor XII deficiency:-
• Usually cause no bleeding
Acquired coagulation disorders

More common than inherited disorders.


Includes :
 Vitamin K deficiency.
 Liver disease.
 Coagulation disorders caused by
antibodies.
.
VITAMIN K DEFICIENCY

Vitamin K is a fat soluble vitamin

 Obtained from green vegetables and bacterial synthesis


in the gut
 Important on coagulation factors II, VII, IX and X and on
proteins C and S.
 Without it, these factors cannot bind calcium.
 Biliary obstruction.
 Malabsorption of vitamin K.
 Vitamin K antagonist drugs.
LIVER DISEASE

Biliary obstruction results in malabsorption of


vitamin K and therefore :-
Decreased synthesis of factors II, VII, IX and
X.
Complications associated with bleeding disorder

 Bleeds beneath the skin can cause large bruises that take
time to heal.
 Bleeding in certain areas,the eye, head, brain, throat or
gastrointestinal tract can be life threatening and require
immediate attention.
 Joint Damage: This occurs when there is repeated bleeding
into the joints. Joint bleeds cause pain and discomfort that
can lead to chronic swelling and deformity.
 Inhibitors: Inhibitors are antibodies that the body develops
because it sees the infused factor as a foreign substance that
needs to be destroyed. Antibodies are proteins that eat up the
activated factor before it has time to stop the bleeding
Complication cont,

 Viral infection: Factor products are considered


safe, however patients still run the risk of a new
virus being introduced.
 Emotional and psychological effects: including
stress, sadness and even depression. It’s
important to know that these feelings are normal,
and there are ways to help patients deal with the
emotional stress of the disease.
Treatment

 Treatment depends on the type of disorder. It may


include:

 Clotting factor replacement:-


 Treated by replacing missing blood clotting factors,this is
called clotting factor replacement therapy. Clotting factors
are replaced by injecting a clotting factor concentrate into
a vein.
 It can prevent severe blood loss and
complications from bleeding such as damage to
muscle, joints, and organs.
.
Treatment cont,

 Fresh frozen plasma transfusion


One unit of FFP is the plasma taken from a unit of whole blood. FFP is
frozen within eight hours of collection. FFP contains all coagulation factors
in normal concentrations. Thawed plasma may be transfused up to 5 days
after thawing and contains slightly decreased levels of Factor V (66+/-9%)
and decreased Factor VIII levels (41+/-8%).
Treatment cont,

 Platelet transfusion:-
The platelets are run through a drip and into your
bloodstream. The transfusion usually takes 15-30
minutes and can usually be done at an outpatient
clinic,and all donated platelets are tested for
infections or viruses.
Treatment cont,
 Tranexamic Acid
It is an antifibrinolytic agent, used for prevention of excessive
menstrual bleeding. It is also used for prevention of bleeding due to
other causes such as peptic ulcer,
 Factor IX
It is a synthetic protein,prescribed for prevention of hemorrhagic
episodes in patients with hemophilia B.
 Fibrinogen Concentrate
It is a hematological agent, prescribed for acute bleeding episodes in
patients with congenital fibrinogen deficiency.
 Antihemophilic factor.

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