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Bleeding

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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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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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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3. Coagulation phase
Through two separate pathways the
conversion of fibrinogen to fibrin is
complete.

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4. Fibrinolytic phase
Anti clotting mechanisms are
activated to allow clot
disintegration and repair of the
damaged vessel

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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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Laboratory evaluation

Platelet Bleeding Prothrombi


count time (BT) n time (PT)

Partial
thrombopl Thrombin
astin time time (TT)
(PTT)

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Platelet
count

Normal 100.000 400.000


cell/mm
Trombocytopenia < 100.000 cell/mm
Mild Trombocytopenia 50.000 - 100.000
cell/mm
Severe < 50.000 cell/mm
Trombocytopenia
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Bleeding Prothromb Thrombin
time in time time
Provides Measures Time for
assessme effective thrombin
nt of ness of to convert
platelet the fibrinogen
count extrinsic fibrin
and pathway A
function Normal measure
Normal value: of
value: 2- 10-15 fibrinolyti
8 minutes seconds c pathway
Normal
value: 9-
13
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So, what cause bleeding
disorders?
Vessels
Vessels
defects
defects
Platelets
Platelets
disorders
disorders
Factor
Factor
deficiencie
deficiencie
ss
Other
Other
disorders
disorders
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Vessels defect
Vitamin C deficiency
Bacterial & viral infections
Acquired & hereditary conditions
Infectious and hypersensitivity
vasculitides
Rickettsial and meningococcal
infections
Henoch-Schonlein purpura (immune)

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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

Echymoses Small, superficial Large, deep


(bruises)
Hemarthrosis Extremely rare Common
(muscle
bleeding)
Bleeding after Yes No
cuts and
scratches
Bleeding after Immadiate
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Usually mild2015 Often severe 20
Platelet
Coagulation

Petechiae, Purpura Hematoma, Joint b


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Petechia
(typical e
of platelet
disorders)

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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 and Liver disease


B Vitamin K
vonWillebrands deficiency/warfarin
disease overdose
Other factor DIC (Disseminated
deficiencies Intravascular
Coagulation)
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Hemophilia A and B
Hemophilia Hemophilia
A B
Coagulation Factor VIII Factor IX
factor
deficiency
Inheritance X-linked X-linked
recessive recessive
Insidance 1/10.000 males 1/50.000 males
Severity Related to factor level
<1% : Severe (spontaneous
bleeding)
1-5% : Moderate (bleeding with
mild injury)
5-25% : Mild
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IDK(bleeding
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Clinical manifestation of
hemophilia
Hemarthrosis (most
common)
Fixed joints
Soft tissue
hematomas (e.g:
muscle)
Muscle atrophy
Shortened tendons
Other sites of
bleeding
Urinary tract
CNS, neck (may be
life-threatening)
Prolonged bleeding
after surgery or
dental extractions
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Treatment of hemophilia

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

Required for synthesis Factors II, VII, IX ,X


Protein C and S

Causes of deficiency Malnutrition Biliary


obstruction Malabsorption Antibiotic
therapy
Treatment Vitamin K Fresh frozen plasma

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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.

Coagulation Prothrombin time Extrinsic/common pathways


Partial thromboplastin time Intrinsic/common pathways
Coagulation factor assays Specific factor deficiencies
50:50 mix Inhibitors (e.g., antibodies)
Fibrinogen assay Decreased fibrinogen
Thrombin time Qualitative/quantitative
fibrinogen defects
FDPs or D-dimer Fibrinolysis (DIC)

Platelet function von Willebrand factor vWD


Bleeding time In vivo test (non-specific)
Platelet function analyzer (PFA) Qualitative platelet dis
and vWD
Platelet function tests Qualitative platelet dis
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Laboratory Evaluation of the
Coagulation Pathways
Partial thromboplastin timeProthrombin time
(PTT) (PT)
Surface activating agent Thromboplastin
(Ellagic acid, kaolin) Tissue factor
Phospholipid Phospholipid
Calcium Calcium

Intrinsic Extrinsic pathway


pathway

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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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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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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