Mucocutaneous Hemorrhage

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Mucocutaneous Hemorrhage
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Definition

• Mucocutaneous hemorrhage is caused by the
  abnormalities of hemostasis(??) and /or
  coagulation(??), characterized by local or
  extensive mucocutaneous hemorrhage derived from
  capillary. The bleeding is usually spontaneous
  or from slight trauma.

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Hemostasis/coagulation

• Hemostasis/coagulation is the body's normal
  physiological response for the prevention and
  stopping of bleeding/hemorrhage.

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Hemotasis

• Primary hemostasis
• ---platelet plug formation at sites of injury
• Secondary hemostasis
• ---plasma coagulation system reaction resulting
  in fibrin formation

Primary and secondary hemostasis are closely
linked
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Vessel wall
Blood platelets
fibrinolysis
Hemostasis coagulation
Coagulation factors

• anti-coagulation substances

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Etiology Pathogenesis

• Vessel wall disorders
• Abnormalities of blood platelets
• Quantitative platelets defects
• Qualitative platelets defects
• Disturbance of coagulation in circulation
• Deficiency of coagulation factors
• Increase of anti-coagulation substances
• Hyperfunction of fibrinolysis(??????)

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Defects on the vessel wall
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Capillaries are made only of endothelium
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Constriction of capillary playing the role of
hemostasis
local vascular constriction
sealing the damaged vascular endothelium
reducing blood flow
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Vascular wall disorders

• Damage to capillary endothelium
• Abnormalities in the vascular subendothelial
  matrix
• Defect of extravascular connective tissues that
  support blood vessels
• Formation of abnormal blood vessels.

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Defects on Capillary Wall

• Hereditary
• Telangioectasia(??????)
• Acquired
• Allergic purpura(?????)
• Senile purpura
• Non- thrombocytopenic purpura
• Severe infection
• Vitamin C deficiency
• Uremia

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• Vitamin C is needed to synthesize
  hydroxyproline(????), an essential constituent of
  collagen

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Cushing's syndrome

• excess production of glucocorticoids
• develop generalized protein wasting
• atrophy of the supporting connective tissue
  around blood vessels
• skin bleeding or easy bruising

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Senile purpura

• Ageing causes a similar atrophy of perivascular
  connective tissues

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• In this situation,BT and CT are normal,but
  capillary fragility test is positive.

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Abnormalities of blood platelets
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Blood platelets function in primary hemostasis

• platelet adhesion and aggregation
• Platelet activation from thromboxane A 2
  further aggregates platelets (to form the
  white thrombus ) and enhances vasoconstriction
  
• platelet secretion activated platelets
  secrete platelet agents, 5-HT and coagulators
  participating in the coagulation process and
  facilitate the clot constriction.

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Red blood cell
Blood platelet
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Some of the products secreted by platelets are
depicted as ADP, adenosine diphosphate(?????) PD
GF, platelet-derived growth factor(?????????)
vWF, von Willebrand factor.
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Generation of thromboxane A2 in platelets and
prostacyclin (PGI2) in endothelial cells.
arachidonic acid(?????) cyclooxygenase(????) end
operoxide(??????) thromboxane(???) prostacyclin(
????)
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Abnormalities of Blood Platelets

• Quantitative platelets defects
• BPC is low, as thrombocytopenia(??????)
  with various causes
• Qualitative platelets defects
• Platelets dysfunction, as thrombasthenia(?????)
  

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Causes of thrombocytopenia

• Primary thrombocytopenia
• Immunologic thrombocytopenia(viral or bacterial
  infections)
• Drug-induced thrombocytopenia
• Hypersplenia(?????)
• Aplastic anemia(???????), leukemia(???)

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Functional platelet disorders

• Congenital
• Thrombasthenia
• Giant platelet syndrome(????????)
• Acquired
• Liver disease
• Uremia
• Drug-induced

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• In platelet defects,bleeding time is prolonged
  and clot retraction is poor.

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Deficiency of coagulation factors
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Coagulation process

• First stage the formation of activated
  thrombokinase(??????).
• Second stagethrombokinase converts
  prothrombin(????) into thrombin(???).
• Final stage thrombin in turn converts
  fibrinogen(?????) into fibrin(????).

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Congenital plasma coagulation defects

• Hemophilia A-dificiency of factor VIII
• Hemophilia B-dificiency of factor IX
• Hemophilia C-dificiency of factor XI
• Deficiencies in factors V, VII, X, and
  prothrombin (factor II)

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Acquired coagulation disorders

• Vitamin K deficiency(II, VII,IX, X)
• Liver disease
• Disseminated intravascular coagulation (DIC)
• Complications of anticoagulant therapy

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Vitamin K deficiency

• Serves as a cofactor in the enzymatic
  carboxylation of glutamic acid(???) residues on
  prothrombin complex proteins (factors II, VII,
  IX, X proteins C and S)
• Plasma levels of all the prothrombin complex
  proteins decrease.

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Vitamin K Deficiency

• Inadequate dietary intake
• Intestinal mal-absorption
• Loss of storage sites due to hepatocellular
  disease

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Coagulation Disorders in Liver Disease

• Decreased production of coagulation
  proteins(II,VII,IX,X), and fibrinogen(factor I)
  and factor V
• Some degree of vitamin K deficiency
• Hypercoagulable" and predisposed to developing
  DIC or systemic fibrinolysis.

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Lab test of coagulation defect

• PT
• PTT
• CT
• platelet count
• fibrinogen determination

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Fibrinolytic system
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(????)
(????)
(???)
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Defects in the fibrinolytic system

• a2 plasmin inhibitor deficiency or plasminogen
  activator inhibitor (PAI) 1
• Secondary fibrinolysis accompanying DIC

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Increase of anti-coagulation substances

• the increase of heparan anticogulants or the
  over-dosage of anticoagulants.

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Types of Mucocutaneous hemorrhage

• Petechia(?????) pinpoint hemorrhage
• lt2mm in diameter
• Purpura(??) gt 3- 5mm in diameter
• Ecchymosis(??) common bruise, gt5mm
• Hematoma(??) local elevation and fluctuation
• Epistaxis(???)
• Bleeding in skin, mucous membrane, joint cavity
  and viscera(??).

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Petechiae
Ecchymosis (Bruise)
Hematoma
Purpura
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Approach to patients with mucocutaneous hemorrhage
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History-taking

• Age,male or female,onset of the disease
• Spontaneous or following trauma
• Past history of bleeding tendency, such as
  bleeding after tooth extraction,
• Liver disease
• A family history of bleeding and bleeding from
  multiple sites, repeated episodes
• A record of drug ingestion

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Physical examination

• Bleeding should be traced over whole body,
  sometimes including urine and stool.
• In addition, the jaundice and the size of liver
  and spleen should be noted.

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Bleeding from a platelet disorder

• Bleeding sites localized to superficial sites
  such as the skin and mucous membranes
• Immediately after trauma or surgery
• Readily controlled by local measures

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Bleeding from plasma coagulation defects

• Bleeding sites in deep subcutaneous tissues,
  muscles, joints, or body cavities
• time hours or days after injury
• Unaffected by local therapy

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Laboratory routine tests

• Blood routine
• Blood platelet count
• Bleeding time (a sensitive measure of platelet
  function)
• Prothrombin time (PT, screens the extrinsic limb
  of the coagulation system)
• Coagulation time
• Bone marrow study
• Coagulation factors determination
• Fibrinogen determination.

Screening tests
Specific tests