Professional Documents
Culture Documents
Disorders
Diyah Candra Anita K.
diyah.candra@yahoo.com
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2015 1
Hemostasis
Hemostasis is a
process which
Vascular
Vascular
causes bleeding phase
phase
to stop, meaning
to keep blood Platelet
Platelet
within a phase
phase
damaged blood
vessel. Coagulation
Coagulation
phase
phase
The opposite of
hemostasis is Hemostasis
Fibrinolytic
Fibrinolytic
hemorrhage. phase:phase
phase
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2015 2
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Normal clotting
Response to vessle injury:
Vasoconstriction to
reduce blood flow
Platelet plug formation
(von willebrand factor
binds damaged vessle
and platelets)
Activation of clotting
cascade with
generation of fibrin
clot formation
Fibrinolysis (clot
breakdown)
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2015 4
Clotting cascade
Normally the
ingredients,
called factors,
act like a row of
dominoes
toppling against
each other to
create a chain
reaction.
If one of the
factors is
missing this
chain reaction
cannot proceed.
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1. Vascular phase
When a blood
vessel is
damaged,
vasoconstriction
results.
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2. Platelet phase
Platelets adhere to the damaged
surface and form a temporary plug
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2015 8
3. Coagulation phase
Through two separate pathways the
conversion of fibrinogen to fibrin is
complete.
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2015 9
4. Fibrinolytic phase
Anti clotting mechanisms are
activated to allow clot
disintegration and repair of the
damaged vessel
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2015 10
Hemostasis
Dependent upon:
Vessel wall integrity
Adequate numbers of
platelets
Proper functioning
platelets
Adequate levels of
clotting factors
Proper function of
fibrinolytic pathway
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2015 11
Laboratory evaluation
Partial
thrombopl Thrombin
astin time time (TT)
(PTT)
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Platelet
count
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Platelet disorders
Trombocytopenia Trombocytopathy
Inadequate number of Adequate number but
platelets abnormal function
Etiology: Etiology:
Drug induced
Uremia
Bone marrow failure
Inherited disorders
Hypersplenism
Other causes:
Myeloproliferative
Lymfoma disorders
HIV Drug induced
Idiopatic
trombocytopenia
purpura (ITP)
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Factor deficiency
(congenital)
Hemophilia
Hemophilia AA 80-85% of all
Hemophiliacs
(Classic
(Classic Deficiency of Factor VIII
hemophilia)
hemophilia) Lab Results - Prolonged
PTT
Hemophilia
Hemophilia BB 10-15% of all
Hemophiliacs
(Christmas
(Christmas Deficiency of Factor IX
hemophilia)
hemophilia) Lab Test - Prolonged
PTT
Von
Von Deficiency of VWF &
Willwbrands amount of Factor VIII
Willwbrands Lab Results - Prolonged
disease
disease BT, PTT
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Other disorders (acquired)
Oral anticoagulants
Coumarin
Heparin
Liver disease
Malabsorption
Broad-spectrum antibiotics
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Clinical Features of Bleeding
Disorders
Coagulation factor
Platelet disorders
disorders
Skin Deep in soft tissues
Mucous membran (joints, muscles)
Site of bleeding (epistaksis, gum,
vaginal, GI tract)
Petechiae Yes No
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Hemarthrosis
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Hemato
ma
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Purpura
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Ecchymosis
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Coagulation factor disorder
Inherited
Inherited bleeding
bleeding Acquired
Acquired bleeding
bleeding
disorders
disorders disorders
disorders
Hemophilia A Hemophilia B
Intermediate purity plasma
products Agent
Virucidally treated
May contain von Willebrand
High purity factor IX
factor Recombinant human
factor IX
High purity (monoclonal)
plasma products
Virucidally treated
No functional von
Dose
Willebrand factor Initial dose: 100 /kg
Recombinant factor VIII Subsequent: 50 /kg
Virus free/No apparent risk every 24 hours
No functional von
Willebrand factor
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Vitamin K deficiency
Source of vitamin K Green vegetables
Synthesized by intestinal flora
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Classification of platelet
disorders
Quantitative Qualitative
disorders disorders
Inherited
Abnormal disorders (rare)
distribution Acquired
Dilution effect disorders
Decreased Medications
production Chronic renal
failure
Increased Cardiopulmonary
destruction bypass
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Laboratory Evaluation of
Bleeding
CBC and smear Platelet count Thrombocytopenia
RBC and platelet morphology ITP, DIC, etc.
Common pathway
Thrombin time
Thrombin
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Fibrin clot 2015 34
Bleeding time and bleeding
5-10% of patients have a prolonged
bleeding time
Most of the prolonged bleeding
times are due to aspirin or drug
ingestion
Prolonged bleeding time does not
predict excess surgical blood loss
Not recommended for routine
testing in preoperative patients
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2015 35
Treatment Approaches to
the Bleeding Patient
Red blood cells
Platelet transfusions
Fresh frozen plasma
Cryoprecipitate
Amicar
DDAVP
Recombinant Human factor VIIa
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2015 36
Approach to bleeding
disorders
Summary
Identify and correct any specific defect
of hemostasis
Laboratory testing is almost always needed to
establish the cause of bleeding
Screening tests (PT,PTT, platelet count) will often
allow placement into one of the broad categories
Specialized testing is usually necessary to establish a
specific diagnosis
Use non-transfusional drugs whenever
possible
RBC transfusions for surgical
procedures or large blood loss
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Thank you for
attention...
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