2. How To Approach A
child with Bleeding
Disorder
DR. KAMRAN AKBAR
PGR PEDIATRICS
UNIT 2

3. Approach includes:
History
Clinical Examination
Laboratory Findings

4. INHERITED DISORDERS ACQUIRED 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

5. HISTORY:
If child bleeds
from superficial
cut with profuse
bleed?
If fever and
neurological
manifestations?
If history of viral
prodrome ?
Drug related
purpura
Meningococce
mia/TTP
If patient taking
aspirin ,
ibuprofen
,antibiotics.
Coagulation
disorder
Platelet Disorder HUS/ITP
If child bleeds
from injury ,
does the
bleeding stop
and resume?

6. If patient is
neonate ,
with history of
traumatic
delivery or
low APGAR.
If patient is
neonate , and
mother took
phenytoin .
If patient
receive
multiple
blood
transfusions in
the past.
If other family members
have similar problem of
abnormal bleeding or if
repeated episodes of
bleeding gums , prolonged
bleeding as a result of
circumcision.
DIC
Vitamin K
Deficiency
Congenital Disorder
Post transfusional
purpura or
alloimunizational
HISTORY: continued

7. Clinical Examination
The examination should determine the presence of :
 petechiae,
 ecchymosis,
 hematomas,
 hemarthroses,
or mucous membrane bleeding.

8. Petechiae Purpura

9. Senile Purpura DIC

10. Look For :
Easy Bruising
Individuals with
disorders of the
collagen matrix
and vessel wall
may have loose
joints and lax skin
associated with
easy bruising
(Ehlers-Danlos
syndrome).
Mucocutaneous
Bleed
Patients with
defects in
platelet-blood
vessel wall
interaction (VWD
or platelet
function defects)
Deep Bleed
(muscle or joints)
Individuals with a
clotting factor
deficiency of
factor VIII or IX
(hemophilia A or
B)

11. Lymphadenopathy +
hepatosplenomegaly
 Leukemia
 Lymphoma
 Infection
Symmetric
Purpura on Legs
and buttocks
 Henoch–
schonlein
Purpura
Ecchymotic
Lesions
Extensive and in
various stages of
revolutions
 Physical Abuse

12. Bleeding Disorders
Vessel Wall
Disorder
Platelet Disorder
Coagulation
Factor
Abnormalities

13. Vessel wall disorder
Acquired Congenital
1. SENILE PURPURA
2. VASCULAR PURPURA
3. HENOCH SCHONLEIN PURPURA
1. HEREDITARY HEMORRHAGIC
TELENGIECTASIA
2. EHLERS DANLOS SYNDROME

14. Ehlers-Danlos syndrome Henoch-schonlein purpura

15. HEREDITARY HEMORRHAGIC
TELENGIECTASIA
HEMOPHILIA

16. Platelet Disorders
Qualitative Quantitative
• THROMBASTHENIA
• BERNARD-SOULIER SYNDROME
• DRUGS(ASPIRIN,TXA2,INDOMATHACIN
• THROMBOCYTOPENIA
• THROMBOCYTHEMIA

17. Coagulation
Factor
Abnormalities
Hereditary Acquired
1. HEMOPHILIA A(factor VIII deficiency)
2. HEMOPHILIA B(factor IX deficiency)
3. von WILLEBRAND DISEASE
4. DISORDERS OF FIBRINOGEN-HEREDITARY
AFIBRINOGENAEMIA
HYPOFIBRINOGENAEMI DYSFIBRINOGENAEMIA
1. DISSEMINATED INTRAVASCULAR COAGULATION(DIC)
2. LIVER DISEASE
3. VIT K DEFICIENCY
4. MASSIVE TRANSFUSION OF STORED BLOOD
5. ACQUIRED INHIBITORS OF COAGULATION
6. HEPARIN OR ORAL ANTICOAGULANT THERAPY
7. RENAL DISEASE

18. 1. Full blood count and blood film
2. Bleeding time
3. Prothrombin time with INR – measure factors II, V,
VII, X.
4. Activated Partial thromboplastin time measures II, V,
VIII, IX, X, XI and XII.
5. Mixing studies
6. Thrombin Time
7. Quantitative fibrinogen assay
8. D-Dimers
9. Biochemical Screen for renal and Liver function tests
10. Platelet aggregation studies

19. CBC complete blood count, F factor PFA platelet function analyzer, PT prothrombin time, PTT activated partial
thromboplastin time, RIPA ristocetin -induced platelet aggregation, vWD von Willebrand
disease, vWF von Willebrand factor, vWF : R Co ristocetin cofactor activity.

20. Interpretation of Lab results:
Factor
VIII
Factor
IX
vWD Thrombocyto
penia
DIC
Complete blood
examination
Normal Normal Normal Decrease
platelets
counts
Dec. Hb
Dec. Platelets
Peripheral film 
Schistocytes
Bleeding time Normal Normal Increase Increase Increase
PT Normal Normal Normal Normal Prolong
APTT Prolong Prolong Prolong Normal Prolong
Mixing studies Correct
PT, APTT
Correct
PT,
APTT
Correct
PT, APTT
Correct PT, APTT
Clotting factors
assay
VIII
deficiency
IX
deficien
cy
vWF
deficiency
-
Thrombin Time - - - - Increase

21. Treatment of common bleeding
disorders.
Bleeding disorder
 Idiopathic thrombocytopenic purpura
 Disseminated Intravascular
coagulation
 Henoch-schonlein purpura
 Platelet function abnormalities
Treatment
 Intravenous immunoglobulins
 Steroids
 Anti D immunoglobulins
 Continuous platelet transfusion (for life
threatening hemorrhage).
 Fresh frozen plasma
 Platelet and packed red blood cell transfusion.
 Steroids
 Platelet transfusion(for life threatening
hemorrhage)

22. Treatment of common bleeding
disorders (continued)
Bleeding disorder
 Von willebrand
disease (vWD)
Treatment
 Desmopressin acetate (DDAVP)