1. Diagnosis Approach of Bleeding in Children ________________________________ Ketut Ariawati Hematologi Onkologi RSUP Sanglah Denpasar

2. Introduction
Bleeding in a child can be a diagnostic challenge because of the wide range of possible causes
Symptoms
 such as bruising and epistaxis occur frequently in children without underlying bleeding disorder
 So determining which child requires further investigation can be difficult
Bleeding disorder can be inherited or acquired
Evaluation
 comprehensive medical and bleeding history, a complete family history, detailed physical examination and selected laboratory tests

3. Normal Hemostasis
Mechanism by which bleeding from an injured vessel is arrested by formation of a thrombus.
Functions
To maintain the blood in fluid state
To prevent clots in intact vessels
To arrest bleeding in injured vessels
Components
Blood vessels
Platelets
Plasma coagulation factors
Fibrinolytic system

6. PRIMARY SECONDARY INJURY VESSEL WALL+PLATELET FORMATION OF PLT PLUG
STAGES OF HEMOSTASIS
INJURY
VESSEL WALL+PLATELET
FORMATION OF PLT PLUG
PRIMARY
ACTIVATION OF PLASMA COAGULATION
FACTORS
FORMATION OF STABLE FIBRIN CLOT
SECONDARY
DISSOLUTION OF FIBRIN CLOT BY FIBRINOLYSIS

7. STAGES OF HEMOSTASIS PRIMARY SECONDARY Platelet & vessel wall mediated
Occurs within seconds of injury
Forms platelet plug
Prevent blood loss from capillary,arterioles and venules
SECONDARY
Coagulation factors mediated
Takes several minutes for completion
Forms stable fibrin plug
Prevents blood loss from large vessels

8. CAUSES OF BLEEDING
1.Vessel wall disorders 2.Platelet disorders: quantitative or functional. 3.Coagulation factor: deficiency or inhibitors. 4.Combination of these.

9. VASCULAR DISORDERS ACQUIRED CONGENITAL SENILE PURPURA VASCULAR PURPURA
HENOCH SCHONLEIN PURPURA
HEREDITARY HEMORRHAGIC TELENGIECTASIA
EHLERS DANLOS SYNDROME

10. PLATELET ABNORMALITIES
QUANTITATIVE
QUALITATIVE
THROMBOCYTOPENIA
THROMBOCYTHEMIA
THROMBASTHENIA
BERNARD-SOULIER SYNDROME
DRUGS(ASPIRIN,TXA2, INDOMETHACIN

11. DISORDERS OF COAGULATION
HEREDITARY
HEMOPHILIA A(factor VIII deficiency)
HEMOPHILIA B(factor IX deficiency)
von WILLEBRAND DISEASE
DISORDERS OF FIBRINOGEN-
HEREDITARY AFIBRINOGENAEMIA
HYPOFIBRINOGENAEMIA
DYSFIBRINOGENAEMIA
ACQUIRED
DISSEMINATED INTRAVASCULAR COAGULATION(DIC)
LIVER DISEASE
VIT K DEFICIENCY
MASSIVE TRANSFUSION OF STORED BLOOD
ACQUIRED INHIBITORS OF COAGULATION
HEPARIN OR ORAL ANTICOAGULANT THERAPY
RENAL DISEASE

12. APPROACH TO A PATIENT OF BLEEDING DIATHESIS
CLINICAL EVALUATION :
History : Age of first manifestation, Family history of bleeding, Spontaneous or after trauma, Time of manifestation after injury, Ease with which bleeding is controlled, Drug history.
Clinical Picture (tabel)
2. LABORATORY APPROACH
First line screening tests Second line specific tests.

13. Finding
Disorders of Coagulation
Disorders of Platelets or Vessels
Petechiae
Rare
Characteristic
Deep dissecting
hematomas
Superficial ecchymoses
Common; usually large and
solitary
Characteristic; usually small and
multiple
Hemarthrosis
Delayed bleeding
Common
Bleeding from
superficial cuts and
scratches
Minimal Persistent
often profuse
Sex of patient
80–90% of inherited forms occur only in male patients
Relatively more common in females
Positive family history
Rare (exc. vWF , hereditary hemorr.
telangiectasia)

14. Table 118–2. Clinical Manifestations Typically Associated with Specific Hemostatic Disorders
Bleeding from stump of umbilical cord and habitual abortions
Afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, factor XIII deficiency
Impaired wound healing
Factor XIII deficiency
Facial purpura in newborns
Glanzmann thrombasthenia, severe thrombocytopenia
Recurrent severe epistaxis and chronic iron deficiency anemia
Hereditary hemorrhagic telangiectasias

15. Table 118–2. Clinical Manifestations Typically Associated with Specific Hemostatic Disorders
Mucocutaneous bleeding
Thrombocytopenias, platelet dysfunction, von Willebrand disease
Cephalhematomas in newborns, hemarthroses, hematuria, and intramuscular, intracerebral, and retroperitoneal hemorrhages
Severe hemophilias A and B, severe deficiencies of factor VII, X, or XIII, severe type 3 von Willebrand disease, afibrinogenemia
Injury-related bleeding and mild spontaneous bleeding
Mild and moderate hemophilias A and B, severe factor XI deficiency, moderate deficiencies of fibrinogen and factors II, V, VII, or X, combined factors V and VIII deficiency, 2-antiplasmin deficiency

17. Stepwise approach

19. ACQUIRED DISORDERS INHERITED DISORDERS Early age of presentation
Family history positive
More sever
Bleeding is the dominant feature
Single factor defect
Later age of presentation
Family history usually negative
Less sever
Clinical picture is dominated by the underlying disorder e.g.DIC
Multiple hemostatic defect

21. HEMOPHILIA

22. HEMOPHILIA
A group of hereditary genetic disorders that impair the body’s ability to control blood clotting or coagulation
Sex-linked X chromosome
Females are almost exclusively carriers of the disorder, and may have inherited it from either their mother or father

23. Causes
Hemophilia A
X-linked genetic disorder involving a lack of functional clotting Factor VIII and represents 90% of hemophilia cases
Hemophilia B
X-linked genetic disorder involving a lack of functional clotting Factor IX and more severe but less common
Hemophilia C
autosomal recessive genetic disorder involving a lack of functional clotting Factor XI

24. CLINICAL FEATURES: No racial predilection
AGE-Bleeding manifestations begin after 6 months of age.
SIGNS: Hematomas, Hemarthroses,Hematuria,GI bleeding, Bleeding from laceration , head trauma,spontaneous intra cranial bleeding. Hemorrhage into joints that causes muscle spasm.

25. Comparison of vWD and Hemophilias
Hemophilia A
Hemophilia B
von Willebrand Disease
Factor VIII coagulant activity
Low
Normal
Low or normal
von Willebrand factor antigen
von Willebrand factor activity
Factor IX
Ristocetin-induced platelet agglutination
Normal, low, or increased at low-dose ristocetin
Platelet aggregation
Treatment
DDAVP* or recombinant VIII
Recombinant IX

26. Inheritance of Hemophilia

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