HEMOSTASIS

and
HEMORRHAGIC
DISEASE
Bambang Permono
Hematology – Oncology Divisions
Pediatrics Department of Medical Faculty of Airlangga University –
Dr. Soetomo Hospital

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 Hemostasis Function:

1. Prevent bleeding

2. Stop the
bleeding

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Pathogenesis of Bleeding

Vascular disorders

Thrombocytopenia.
Platelet dysfunctions

Blood clotting disorder

Fibrinolysis.

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Picture 1.

Blood coagulation
cascade

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 Vascular disorder

Vasoconstriction in refectories
small arteries, arterioles

Blood flow to the wound

Platelet activation
and clotting factors
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Platelet reactions and the formation of
the primary hemostatic occlusion
 Adhesion
 Aggregation

Primary haemostatic plug (unstable)

+ Platelet + Fibrinogen

Obstruction hemostasis (Unstable Fibrin)

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Platelet Function

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Haemostatic response to vessel injury

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 Hemostatic Test
 DL + peripheral blood smear trombocytopenia
 Bleeding time (3-8 minute)  Platelet Function
 Clotting time  Clotting Factors
 PPT (plasma prothrombin time)  extrinsic
pathway
 APPT (activated partial thromboplastin time)
 intrinsic analysis

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Diseases associated with abnormalities
of blood vessels and platelets

 Bleeding vascular abnormalities

 Hereditary hemorrhagic telangiectasia
 Henoch-Schonlein purpura
 Trombocytopenia
 Failure of platelet production (ATP)
 Increased platelet destruction(ITP)
 Abnormalities of platelet function
 hereditary disorders
 abnormalities akuisita
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 Blood Clotting Disorders

 Hemofphilia
 Factor IX Deficiency
 Von Willebrand Disease
 Deficiency of Vitamin K

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 Hemophilia

 Most hereditary blood clotting disorder

 33% of patients have no family history
 The incidence of 1 per 10,000 population
 Spontaneous intracerebral hemorrhage occurs
more frequently
 Dx Lab: APTT, clotting time & frozen test factor VIII
= abnormal

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 Von Willebrand Disease
 Platelet Adhesion abnormal clotting factor VIII activity
with low reply
 Similar incidents or even exceed hemophilia.
 Real primary defect: the reduction of the synthesis of the
main fraction of factor VIII VWF,
 Bleeding is characterized by bleeding after surgery or
forced ruda, mucous membrane bleeding, and excessive
blood loss from superficial cuts and abrasions.

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 Von Willebrand Disease
 Lab Diagnosis :
 Bleeding time extends
 Clotting factor VIII activity levels (VIIIC) low
 Platelet aggregation with ristocetin
imperfect (defective)
 Activity VII: low VWF in plasma of patients

Treatment : Cryopresipitat

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 Deficiency of Vitamin K (VKDB)
 In newborns marked with bleeding on days 2-4
 Diagnosis: PPT & APTT abnormal
 Treatment :
 Prophylaxis: vitamin K1 (konakion) 1 mg i.m.
to all newborns
 In infants who hemorrhage: vitamin K 1 mg
i.m. every 6 hours
 Due to immaturity of the liver cell function,

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