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    Bleeding Disorders

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    kholoud220
    vWF antigen and activity levels are reduced

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    Bleeding Disorders

    Uploaded by

    kholoud220
    154 views24 pages
    vWF antigen and activity levels are reduced

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    bleeding

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    vWF antigen and activity levels are reduced

    Copyright:

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    Download as pptx, pdf, or txt
    154 views24 pages

    Bleeding Disorders

    Uploaded by

    kholoud220
    vWF antigen and activity levels are reduced

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
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    University of Tabuk

    Faculty of Medicine
    Department of Pathology
    Hemostasis depends on three factors:
    Integrity of blood vessel wall
    Platelets
    Coagulation system
    So Excessive bleeding can result from:
    - Increased fragility of vessels
    - Platelet deficiency or dysfunction
    - Defect in coagulation
    Bleeding time
    Prothrombin time (PT).
    Partial thromboplastin time (PTT).
    Platelet count
    Measure the levels of specific clotting
    factors, fibrinogen,.
    Nonthrombocytopenic purpuras
    Causes:
    1. Infections
    2. Drug reactions
    3. Vitamin C deficiency
    4. Hereditary
    Common but do not usually cause serious
    bleeding problems
    Petechiae and purpura in the skin or mucous
    membranes
    The platelet count, the bleeding time, PT,
    and PTT are normal in bleeding caused
    by decreased integrity of BVs
    An abnormal decrease in the number of
    platelets below 100,000 /L
    Causes:

    1. Decreased platelet production


    2. Increased destruction
    - Immunologic: primary &secondary ITP,
    drugs, infections
    - Nonimmunologic: DIC, prosthetic heart
    valves
    3. Sequestration : In hypersplenism
    Bleeding resulting from thrombocytopenia is
    associated with normal PT and PTT.
    Caused by autoantibodies directed
    against platelet membrane.
    Types:

    1. Primary or idiopathic
    - Acute immune thrombocytopenic
    purpura
    - Chronic immune thrombocytopenic
    purpura
    2. Secondary: in SLE
    Bleeding occur spontaneously or following
    trauma.
    Skin is the most common site of
    haemorrhage
    Haemorrhage may in the form of petechiae
    or ecchymoses, or both.
    Bleeding from mucous membrane is
    common.
    Epistaxis, gum bleeding, menorrhagia,
    melena may also occur.
    Petechiae Ecchymosis (bruises)
    Thrombocytopenia
    Bleeding time (BT): prolonged

    PTT & PT: Normal

    Antiplatelet antibody (IgG)

    BM: Megakaryocytes are increased in


    number.
    Treatment:
    1. Glucocorticoids
    2. Splenectomy
    Physiology:
    Coagulation system constitutes the third
    important component of the haemostatic
    process.
    Blood coagulation pathways are divided into
    extrinsic and intrinsic pathway.
    Coagulation factors of intrinsic pathways are
    XII, XI, IX and VIII.
    Tissue factor (thromboplastin) and VII
    constitute the extrinsic pathways.
    Clotting Mechanism

    INTRINSIC EXTRINSIC
    Collagen Tissue Thromboplastin
    (tissue factor)
    XII
    XI (III)
    IX VII
    VIII

    V Ca++ FIBRINOGEN
    (I)

    PROTHROMBIN THROMBIN
    (II) FIBRIN
    Acquired: Usually multiple factors are
    involved
    - Vitamin K deficiency
    - Liver disease
    Hereditary: Usually single factor is
    involved
    - VIII: hemophilia A
    - IX: hemophilia B
    - von-Willebrand disease
    X-linked recessive disorder.
    Age: Infant and children.
    Sex: male.
    Bleeding manifestations:
    Hemarthrosis
    Recurrent bleeding into the joints leads to
    deformities
    Tendency for bruising and massive hemorrhage
    after trauma or operative procedures
    Hemorrhage from mouth, gums, lips, and
    tongue.
    Petechiae are absent.
    X-linked recessive
    Factor IX deficiency produces a disorder
    clinically similar to factor VIII deficiency
    (hemophilia A)
    Bleeding time: Normal.
    Clotting time: Prolonged.
    PTT: prolonged
    PT: Normal
    Platelet count: Normal
    Confirmatory test:
    - Factor VIII and IX assay:
    - In hemophilia A: factor VIII is decreased.
    - In hemophilia B: factor IX is decreased.
    vW bridges between platelets and sub-
    endothelial collagen tissue
    Stabilize factor VIII in circulation
    Localize factor VIII to site of vessel injury
    An autosomal dominant disease.
    Affects both sexes.
    Most common inherited bleeding disorder
    Manifested with bleeding on skin mucous
    membrane.
    Patients also present with deep-seated
    haemorrhages caused by factor VIII
    deficiency.
    Bleeding time: Prolonged due to defective
    platelet adhesion to subendothelial tissues
    Clotting time: Prolonged
    PTT: Prolong
    PT: Normal
    Platelet count: Normal
    Factor VIII assay: Reduced

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