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Soebandiri

Dept of Medicine, Division of Hematology


Airlangga University School of Medicine
Surabaya

To safeguard that the blood should not decreased in


amount drastically by blood loss/bleeding, this is
done by the formation of thrombus. This function
is called Prothrombotic function.
If this goes unimpeded the whole blood will clot, the
individual will die.
1.

2.

To keep the blood in the blood vesels always in fluid


state, so that it can flow freely to carry nutrients to
the whole body; the function is called anti
thrombotic function which always keep the
prothrombotic function in good balance

a.

The THROMBOCYTE (8 functin) and the


VASCULAR system called : Primary or initial
hemostasis; the end product is a platelet plug
which will stop the bleeding immediately but
temporarily

b.

The PLASMA COAGULATION FACTOR (I-XIII) also


called the secondary hemostasis; the end
product is a stable fibrin which enforce the
primary platelet plug to become a stable and
strong thrombus to stop the bleeding
permanently.

a.

The ANTICOAGULATION subsystem; the


function is the prevent thrombosis (AT-III,
protein C and S)

b.

The FIBRINOLYTIC system who will lyse the


already formed thrombus (plasmin and its
precursor)

Four systems as components of hemostasis


Trombosis

Pendarahan
I

II

III

IV

H
I

K
P

A
K

F
i

Trombosis

H.I. = Hemostasis Primer (Initial)A.K. =


= Vaskular
= Trombosit
K.P. = Koagulasi Plasma
Fibrinolisis
(PRO) TROMBOSIS

Pendarahan

Sistim Anti-Koagulasi
- Alamiah
- Ditimbulkan
F.i.
=
Sistim
ANTI-TROMBOSIS

For the S1 Medical Students only the bleeding


caused by the deficiency of prothrombotic
function must be known, whereas other causes
are nice to know
The bleeding tendency caused by the deficiency of
the prothrombotic forces will be discussed here
and called the Hemorrhagic Diathesis
Hemostasis (+thrombosis) is kept in balance by a
command-post located in the Endothelial cell
The E cells keep the balance by secreting
stimulator as well as inhibitors to the 4
subsystem

To the primary hemostasis


Stimulators : serotonin, VWF
Inhibitor
: NO; protacycline
b. To the secondary hemostasis
Stimulators : Tissue factor
Inhibitor
: Tissue factor pathway inhibitor (TFPI)
c. To the anticoagulation subsystem
Stimulators : Heparan sulfate
Inhibitor
: Thrombomodulin
d. To the fibrinolysis subsystem
Stimulators : tPA
Inhibitor
: PAI
a.

Ax + History of Illness :
Local bleeding : ask local trauma & surgery
DH is a systemic disease multiple sites
bleeding e.g :
Skin
: petechiae, echymosis
Nose
: epistaxis
Mouth : gum bleeding
Uterus : poly/hypermenarrhoe, etc

Theory of Hemostasis

intrinsik

Ekstrinsik

Trombosit +
Vaskuler

(fXII

fXIIa

out

fXI

fXIa
Tissue Factor
fIX

Block

fIXa
(-)

fVII

TFP i

fV III
fX

Antikoagulasi

Thrombin
Fibrin

fXa
pfIII
fV
Prothrombin
fibrinogen

fXIII
Fibrin stabil
FDP
= jalur revisi
= jalur kaskade (lama)

Fibrinolisis

= jalur fibrinolisis

Family Anamnesis

Physical Exam :
Search carefully wether there is multiple sites
bleeding, are there other disease causing
secondary bleeding; e.g Acute leukemia, aplastic
anemia, etc
Laboratory Exam :
a. Routine lab test to look for other diseases
b. Hemostasis lab test

Screening test : Rumpel Leede Test (RL), Bleeding


Time (BT), Thrombocount (TrC), Clotting Time
(CT) or Plasma Recalcification Time (PRT) and
Clot Retraction Test (CR).
These test will enable us to separate bleeding due to
vascular abnormality, platelet abnormality or
coagulation abnormallity
I.

II.

Special Hemostasis Test (confirmatory test)

Platelet function tests; factors assay e.a. tests; is


better done by a specialist in Hematology
(Consultation) also for treatment
As an example we will discuss in short :
a. ITP = Immunologic Thrombocytopenia Purpura
b. Hemophilia

Definition :
ITP is disease caused by thrombopenia due to
immunologic process usually due to auto
antibodies against platelet with bleeding
manifestation from light till severe.
If only the platelet is involved it is called primary
ITP. If other cells are also involved called
secondary ITP

Spontaneous bleeding occur when platelet count


<5000/cmm
Multiple sites bleeding
Thrombopathia pattern of screening test
No other cellular abnormality (WBC nor RBC)
Cause of death ICH

Therapy :
Immuno suppression e.g steroid preferably by a
Hematologist (specialist)
Platelet transfusion if there is overt bleeding

Definition : Hemophilia A/B is a hereditary


recessive X linked deficiency of factor VIII/IX in a
diseased patient
Clinical Manifesttion :
Bleeding since childhood, usually delayed type
after tooth extraction, circumcission etc;
hemarthros
Family anamnesis +
Coagulopathy pattern of screening test
Decreased VIII/IX level (Assay)

F VIII/IX infusion preferably by a hematologist

Many products can be considered


Fresh Frozen Plasma
Cryoprecipitate
Plasma derived F VIII/IX
Recombinant product (genetic engineering)

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