KP 2.2.3.

HAEMORRHAGIC DIATHESIS
RUDY AFRIANT
SUB DIVISI HEMATOLOGI & ONKOLOGI MEDIK
BAGIAN ILMU PENYAKIT DALAM
FAKULTAS KEDOKTERAN UNIVERSITAS ANDALAS
NOVEMBER 2017
BLEEDING

Normal : Trauma, menstruation, partum, surgery,

dental extraction.

Abnormal : Ptheciae, purpura, ecchymosis, hematoma,

epistaxis, ginggival bleeding, hematemesis, melena,
menometrorrhagia.
 ABNORMAL BLEEDING ???

COAGULATION
VASCULAR

PLATELETS
 1. The primary haemostatic system

Primary haemostasis
Platelet aggregation

trombosit
Adhesion
endothelial cells
Activation
sub endothelial tissue

Vascular Aggregation
injury
White clot

Formation of
: platelet plug

exposed sub
endothelial tissue
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Bleeding Disorder caused by Platelet

VWD Glanzmann
Thrombasthenia

Thrombositopenia
Differential Diagnosis

Pseudothrombocytopenia

Decreased Production

Increased Destruction

Sequestration
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 2. Secondary haemostasis

Prothrombin Thrombin Activation of the coagulation

Factor Xa cascade leads to generation of
thrombin and, in turn, fibrin
Intrinsic Fibrinogen Fibrin
Pathway Extrinsic
(colagen) Pathway (TF)

Coagulation cascade
leads to clot formation

Clot
growth
Fibrin threads

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APTT PT
 Hypocoagulability:
- Bleeding diathesis caused by coagulopathy
- Excessive bleeding and a lack of clotting
Acquired Genetics/ Hereditary
Medications:
Anticoagulation: warfarin, UFH, LMWH, Frequent cases
fondaparinux
Thromboslytics: streptokinase, urokinase. Hemophilia A, B
tPA (alteplase, reteplase, tenecteplase)
Antiplatelet agents: aspirin, clopidogrel Von Willebrand disease
Diseases:
Liver failure
Rare cases
Vitamin K deficiency Bernard-Soulier syndrome
Disseminated intravascular coagulation (DIC)
Haemotoxic venom from certain species Glanzmann's
of snakes
Dengue hemorrhagic fever (DHF), Dengue
thrombasthenia
Shock Syndrome (DSS)
Leukemias
Autoimmune: inhibitors to coaugulations (F VIII
inhibitor), Antiphospholipid syndrome (APS)
 Other terms of DIC
Overt DIC = decompensated DIC:
- a state in which the vascular endothelium and its components have
lost the ability to compensate and restore homeostasis in response to
injury
- the result is a progressively decompensating state that is manifest
as thrombotic multiorgan dysfunction and / or bleeding

Non-overt DIC = compensated DIC:

- a clinical vascular injury state that results in great stress to the
hemostatic system, the response to which, for the moment, is sufficient
to forestall further rampant inflammatory and hemostatic activation
 Diagnostic approach
of hemorrhagic diathesis

Clinical manifestations
Laboratory diagnosis
Imaging diagnosis : CT scan
 Clinical symptoms Disorders
Wiskott-Aldrich syndrome, where they
may resemble a few bruises
Petechiae (red spots) Acute leukemia
Chronic leukemia
Vitamin K deficiency

Acute leukemia
Purpura and ecchymoses Chronic leukemia
Vitamin K deficiency

Wiskott-Aldrich syndrome, especially in

Blood in stool infancy
Acute leukemia

Wiskott-Aldrich syndrome
Bleeding gingiva(gums) Acute leukemia
Chronic leukemia

Prolonged nose bleeds Wiskott-Aldrich syndrome

 Complications of coagulopathies, some of them caused by their treatments
Bleeding as a complication of the diseases
Disorders
or their treatment
Soft tissue bleeding, e.g. deep-muscle bleeding, Hemophilia
leading to swelling, numbness or pain of a limb Von Willebrand disease
Joint damage, potentially with severe pain and even Hemophilia
destruction of the joint and development of arthritis Von Willebrand disease
Retinal bleeding Acute leukemia

Transfusion transmitted infection, from blood

Hemophilia
transfusions that are given as treatment.

Adverse reactions to clotting factor treatment. Hemophilia

Anemia Von Willebrand disease

Von Willebrand disease

Exsanguination (bleeding to death) Acute leukemia
Vitamin K deficiency

Cerebral hemorrhage Wiskott-Aldrich syndrome

 Hemorrhagic diathesis Bleeding Algorithm
Approach

Overt Visible bleeding Non overt Non visible bleeding

Mild Moderate Profuse

bleeding bleeding
Intra cavity Occult Pale decreased
bleeding bleeding: bleeding: Hb
-Purpura -Hemorrhoid - Hematemesis
- Cranial - Upper and
-Epistaxis - Menometro -Melena - Pleural lower GIT
-Gum rhagi -Hematoschezia - Pericardi Faeces
bleeding -Traumatic al benzidine Bleeding ?
- Abdomin test Sites of bleeding
Class I, Class II, Class III, Class IV ???
al - Urinary
Bleeding
- Pelvic tract
- Douglas microhem
Can be Fatal cavity aturia
 Prediction of Blood Loss based on Early Clinical Findings in
Male with Body weight of 70 Kg(2)

Parameters Class I Class II Class III Class IV

Blood loss (ml) to 750 750 15000 1500 2000 > 2000

Blood loss to 15 % 15 30 % 30 40 % > 40 %

(% volume )

Pulse rate (minute) < 100 >100 > 120 > 140

Blood pressure (mmHg) normal normal decreased decreased

Resp. rate (menit) 14 20 20 30 30 40 > 35

Urine prod. (ml/jam) > 30 20 30 5 15 tidak ada

CNS/mental restless - restless - restless & restless &

status mild moderate confused lethargic

Fluid replacement cristaloid cristaloid cristaloid & cristaloid &

(based on 3:1 rule) blood blood

Based on: The American College of Surgeon Committee on Trauma(2)

 Laboratory tests of hemostasis
Rumple Prothrombin Activated partial Fibrinogen
Leeds/ time / PT thromboplastin time /APTT degradation
Torniquet products
test Primary hemostasis: Fibrinogen /FDP
- blood vessels
Platelet (particularly: Second stage: Third stage:
count endothelial layer) - plasma procoagulant proteins - repair of vascular damage D-dimer
- cellular blood (clotting or coagulantion factors) results in a return to
Bleeding elements (particularly: - formation of stable fibrin clot the normal state
time/ BT platelets)
- culminates in the
Platelet formation of platelet
plug 2 control process
function
assay / PFA
natural anti
coagulants system
fibrinolysis Plaminogen
- inhibitor proteins
Protein C system Control Plasmin
Control - endothelial-cell-
Protein S based mechanisms system
system
AT III

( important in limiting clot formation to areas of vascular injury)

 Partial
Prothrombin time Bleeding time
thromboplastin Platelet count
Condition (PT) (BT)
time (aPTT)

Vitamin deficiency or Normal or mildly

Prolonged Unaffected Unaffected
warfarin prolonged

Disseminated
intravascular Prolonged Prolonged Prolonged Decreased
coagulation (DIC)

Prolonged or
Von Willebrand disease Unaffected Prolonged Unaffected
unaffected

Haemophilia Unaffected Prolonged Unaffected Unaffected

Aspirin Unaffected Unaffected Prolonged Unaffected

Thrombocytopenia Unaffected Unaffected Prolonged Decreased

Liver failure, early Prolonged Unaffected Unaffected Unaffected

Radio-imaging in bleeding
Intra cranial bleeding Intra abdominal bleeding
X-ray computed tomography CT scan
(CT)
Magnetic resonance imaging Intrathoracic bleeding
(MRI)
CT scan
Positron emission tomography
(PET)
Angiography
TREATMENT

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