BLEEDING

DISORDERS
 Bleeding Disorders
*Are characterized clinically by abnormal bleeding.

Which may be :
- Spontaneous
- Become evident after an even * truma * surgery
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Causes of bleeding disorders: include:
1-abnormalities of vessels
( increased fragility of the vessels )
2- disorders of platelets
3- coagulation disorders
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I-Abnormalities of vessels
Etiology :
- Associated with severe vitamin c deficiency (scurvy )
- Vasculitis

Clinical Features :
- Spontaneous petechiae .
- Ecchymoses in skin and mucous membranesm( with minor trauma )
II- Disorders of plateles : include
1- deficiency of platelets ( thrombocytopenia )
2- defects in platelets function: include :
A- acquired defdcts :
as in uremia , after aspirin ingestion.
B- inherited defects :
as in Von Willebrand disease .
- The PT and PTT are Normal , but the Bleeding time is
Prolonged.
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III _Coagulation Disorders :
- due to deficiency of certain coagulation factors .
 Coagulation cascade.

Both the extrinsic and intrinsic coagulation systems

use the final common pathway for the formation of a
fibrin clot.
 Test used in initial evaluation of patients
With bleeding disorders
1- platelet counts:
- normal rang: 150,000 400,000cells/mm3.
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2- bleeding time:
- evaluates platelet function up to the temporary
platelet plug
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3- prothrombin time (PT):
- tests the extrinsic end common coagulation pathways:
- factors evaluated: VII,X,V,Prothrombin,Fibrinogen.
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4- partial thromboplastin time ( PTT):
- tests the intrinsic & common coagulation pathways:
- factors evaluated :XI,IX,X,VIII, Prothrombin,Fibrinogen.
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5- specific clotting factors measurement :.
 Thrombocytopenia
Definition :
- dened as platelet count of 100 000 cells/uL or less
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Etiology:
The major causes of thrombocytopenia are:

1- decreased production of platelet

2- increased destruction of platelet :
A- Immunologic destruction:
e.g . immune thrombocytopeic pupura (ITP) , drugs

B- Non immunologic destruction:

e.g . thrombotic thrombocytopeic pupura , DIC
3 sequestra on in the spleen :e.g hypersplenism .
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Clinical features:
- increased risk of post-traumatic bleeding & spontaneous bleeding
- petechiae or large ecchymoses in skin
- mucous membranes of GIT & urinary tracts
- markedly decreased platelet counts larger hemorrhages into the CNS

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Laboratory Findings: - The PT & PTT are NORMAL
*but the bleeding time is prolonged
 Immune thrombocytopenic purpura( ITP)
Etiology & pathogenesis:
- Antiplatelet antibodies (IgG) directed against platelet .
membrane antigens
- the antiplatelet antibodies are made in the spleen
- IgG-coated platelets are Destroyed in the spleen by the
macrophages.
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Classification :
- ITP is classified into either :
1- secondary ITP ( of known cause )
2- primary ITP ( Idiopathic) :
Two clinical subtypes of primary ( idiopathic) ITP :
1- acute primary ITP :
- self limited disorder
- commonly seen in childern following a viral infection .
2- chronic primary ITP :
- common disorder
- usually seen in adult females between 20 40 years
- May be a manifestation of systemic lupus erythematosus .
Clinical features :
-petechiae , ecchymoses (bruises),
-menorrhagia , epistaxis (nosebleeds), gum bleeding
-Hemorrhages after minor rtauma.
-The spleen is usually Normal in size.
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Petechiae in idiopathic thrombocytopenic purpura

Show pinpoint hemorrhages , a sign of platelet dysfunction , in the
Skin over the thorax and shoulders .
Laboratory diagnosis:
-Decreased platelet count below 100 000
-prolonged bleeding time.
-normal PT and PTT.
-peripheral blood smear shows thrombocytopena
-Bone marrow biopsy: increased number of megakaryocytes (DD
from secondary ITP due to marrow failure )
- Tests for detection of antiplatelet antibodies.
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Treatment :
- Corticostteroide :
- Immunoglobulin therapy :
- Splenectomy : complete remission in most patients.
 Coagulation Disorders
Factor VIII Deficiency
( hemophilia A, classic hemophilia )
- Hemophilia A is the most common hereditary disease
associated with serious bleeding .
- It is an X-linked recessive disorder
Cauesd by : deficiency of Factor vIII
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Clinical features :
- Predominately affects males
- Symptoms dependent on the degree of deficiency
- Spontaneous hemorrhages ( or after trauma ) into joints
( hemarthrosis )
- Recurrent bleeds into the joints progrssive deformities
- Easy briising and hematoma formation after minor trauma
- Severe prolonged bleeding after surgery or lacerations
- Petechiae are characteristically Absent
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Laboratory diagnosis of hemophilia A
1- Normal platelet count and bleeding time
2- Normal PT and Prolonged PTT
3- Specific VIII assays are required to confirm the diagnosis
Treatment : Factor VIII concentrate
 Factor IX deficiency
( hemophilia B ,christmas disease )

- Caused by: deficiency of factor IX

- It is an x-linked recessive disorder
- Clinically identical to hemophilia A , but much less common
Laboratory diagnosis :
- The bleeding time is Normal.
- The PTT is prolonged and
- Specific assays of factor IX for diagnosis
-Treated by infusion of recombinant factor IX
 Von Willebrand Disease
- Autosomal dominant disorder
- Most vommon hereditary coagulation ( bleeding ) disorder
Characterized by:
- Decreased quantity of Von Willebrand factor ( vWF) ,
and its deficiency causes a secondary decrease in factor VIII levels ( but
not clinically )
Thus , the patients have a compound defect involving the plateler
function and the coagulation pathway .

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structure and function of factor VIII - von Willebrand factor ( vWF) complex Factor VIII and vWF
are synthesized in the liver and in Endothelial cell ,respectively .The two circulate as a complex
in the circulation .vWF is also present in the subendothelial matrix of normal bllod vessels
.Factor VIII takes part in the coagulation cascade by activating factor X. vWF causes adhesion
of platelets to subendothelial collagen through the platelet receptor ( Gplb).Ristocetin
activates Gplb receptors in vitro and causes platelet aggregation if vWF is present
Clinical features :
- Spontaneous bleeding from mucous membranes
- Prolonged bleeding from wounds
- Menorrhagia in young females
Laboratory diagnisis ;
- normal platelet count .
- prolonged bleeding time ( due a platelet adhesion defect)
- normale PT with often a Prolonged PTT
- Abnormal platelet response to ristocetin ( adhesion defect)
is an important diagnostic test .

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