This is appt presentation done by me and my colleagues Bahaa Alsayed and Anas mohammed ( agroup of medical undergarduates , school of Medicine, Ain-shams university , Cairo , Egypt ) ...This work was presented at the end of our Pediatrics clinical round .. I Hope every one to get the best out of the presentaion ..Any commentaries are even more appreciated :)

1. Objectives :

2. Hemostasis
BV Injury
Thromboxane Platelet
A2 ADP Phospolipid
Serotonin
Platelet
Activation
Stable Hemostatic Plug

4. Vascular Platelet
defect defect

5. Primary Hemostatic Disorders
Vascular defect :
 Congenital : Hereditary hemorrhagic telangectasia
 Acquired :
 Vitamin C deficiency (scurvy)
 Connective tissue disorders
 Henoch schonlein purpura
 Infections : Meningiococcemia
Platelet defect :
  platelets (thrombocytopenia) :
1. Decreased platelet production
2. Abnormal distribution
3. Increased destruction
4. Decrease production and increased destruction
 Platelet Dysfunction ( Thrombasthenia ) :

6. Primary Hemostatic Disorders
  platelets (thrombocytopenia)
:
 Congenital : TAR Syndrome , Fanconi anemia
 Acquired : BM cavity abnormality , Stem cell abnormality
 Splenomegaly
Giant hemangioma
 Immune mediated : (ITP, drugs, infections : EBV, HIV)
 Non immune mediated : (DIC , TTP , HUS )
 Congenital infections

7. Primary Hemostatic Disorders
Platelet Dysfunction ( Thrombasthenia ) :
 Glanzmann’s (normal platelet morphology , defect of platelet
aggregation )
 Bernard sloer ( qualitative and mild quantitative platelet disorder
, failure of platelet adhesion)
 platelets (thrombocytopenia)
Von willebrand disease ( quantitative or qualitative deficiency of
vWF molecule which
: - binds to exposed subendothelial collagen
- mediates initial platelet adhesion
 Aspirin
 Renal failure
 Heparin in high doses

9. Secondary Hemostatic Disorders
Coagulopathies :
 Hemophilia A (factor VIII defeciency , classic hemophilia )
 Hemophilia B (factor IX defeciency , christmas disease )
 Hemophilia C (factor XI defeciency )
Von willebrand disease ( Vascular emophilia )
Congenital afibrinogenemia
 Vit. K defeciency
 Liver disease
 DIC ( consumptive coagulopathy )
 Anticoagulants : Oral anticoagulants – Heparin

10. • Age
• Sex
• Trauma
• Past bleeding history (tooth ,circumcision)
• Distribution
• Associated symptoms ( Fever and weight loss
), Abdominal pain and arthritic pain ( HSP)
• Review of other systems
• Medications
• Family history of bleeding

11. Typical Presentations

12. Typical Presentations cont.

13. Clinical approach in making a diagnosis
Clinical Examination
• Petechiae ( less than 2 mm)
• Purpura ( more than 2 mm and less than
20 mm)
• Ecchymosis ( more than 20 mm)
• Joint bleed
• Hepatosplenomegaly
• Lymphadenopathy
• Evidence of underlying liver disease

14. Petechiae Purpura
Ecchymosis Hemarthrosis

15. Vascular causes :
Vascular causes :
Platelet defect Coag.fact defect
Site of bl. Skin and mm. Deep in soft
Petechiae Yes No
Echymosis Small , superficial Large , deep
Hemarthrosis Rare Common
Hematomas Rare Common
Bl.after cuts Early Late
Bl.post surgery Early Late

16. Clinical approach in making a diagnosis
General Specific
tests tests
• CBC • Blood film
• Platelet f. tests
• BT • Fibrinolysis tests
• Cl. factor assay
• PT ,PTT • Mixing test
• vWF assay
• TT • PFA

17. CBC Blood Film
PT
PTT

18. Data Interpretation
• Liver disease
• Warfarin therapy
• Vit. K def.
• DIC ( early )
• Factor 7 def.
• Factor 7 inhibitor
Dose the patient has factor def. ?
Do mixing tests
Corrected Not corrected
Factor def. Inhibitors or lupus anticoagulant

19. Data Interpretation
• Heparin therapy
• Factor 8 , 9 ,11 , 12 def.
• vWD
• Factor inhibitor
• Lupus anticoagulant
• Common pathway def.
• Heparin or warfarin overdose
• Late liver disease
• DIC
• Factors inhibitors
• Primary fibrinolysis
• Dysfibrinoginemia

20. Data Interpretation
Liver disease DIC Primary
fibrinolysis
Factor 8 normal Factor 8 low Platelet normal
D- dimer test normal Abnormal D - D- dimer test
dimer normal

21. Data Interpretation
Consider evaluating for:
• Platelet disorder
• Mild factor deficiency
• Factor XIII
• Monoclonal gammopathy
• Abnormal fibrinolysis
• a2 anti-plasmin deficiency
• Vascular disorders
• Dysfibrinogenemia

22. Data Interpretation
Normal PT , PTT
Think of platelet
Normal platelet Thrombocytopenia
• Do PFA-100 Look for the
cause
Think of
• vWD
• Factor 13 def.
• Vasculopathy

24. Current treatment strategies
Treatment Indication
Platelet concentrate Low pt , platelet dysfunction
Fresh frozen plasma “ FFP ” Multi-factor coagulopathy
Factors V, X (XI, XIII) deficiency
Cryoprecipitate Low fibrinogen, FXIII deficiency
Platelet dysfunction
Prothrombin concentrate Multiple Vitamin K deficiency
Liver Disease
Aminocaproic Acid Excessive fibrinolysis
DDAVP Platelet dysfunction
Factor concentrate
-rFVIIa FVII deficiency
-FVIII FVIII deficiency
-FIX FIX deficiency