Clinical Approach To A Bleeding Child
Overview of haemostasis
Review the most common bleeding Conditions
Clinical approach in making a diagnosis• History • Examination • Investigations
Current treatment strategies
Objectives :
HemostasisBV Injury
Platelet“Aggregation”
PlateletActivation
Blood Vessel “ Constriction’’
Coagulation Cascade
Stable Hemostatic Plug
Fibrin FormationRed
uced
Blood flow
Tissue F
acto
r
Primary hemostatic plug
Neural
Serotonin
Platelet Phospolipid
Thromboxane A2 ADP
Coagulation Cascade
Most common bleeding Conditions
Primary Hemostatic Disorders
Secondary Hemostatic Disorders
( Coagulaopathies )
Vascular defect
Platelet defect
Primary Hemostatic Disorders
Congenital : Hereditary hemorrhagic telangectasia Acquired :
Vitamin C deficiency (scurvy) Connective tissue disorders Henoch schonlein purpura Infections : Meningiococcemia
Vascular defect :
platelets (thrombocytopenia) :
1. Decreased platelet production2. Abnormal distribution 3. Increased destruction 4. Decrease production and increased destruction
Platelet Dysfunction ( Thrombasthenia ) :
Platelet defect :
platelets (thrombocytopenia) :
Primary Hemostatic Disorders
1- Decreased platelet production :Congenital : TAR Syndrome , Fanconi anemiaAcquired : BM cavity abnormality , Stem cell
abnormality
2-Abnormal distribution : Splenomegaly
Giant hemangioma
3- Increased destruction : Immune mediated : (ITP, drugs, infections :
EBV, HIV)Non immune mediated : (DIC , TTP , HUS )
4- Decreased production and increased destruction :Congenital infections
Platelet Dysfunction ( Thrombasthenia ) :
Primary Hemostatic Disorders
platelets (thrombocytopenia) :
A- Hereditary :Glanzmann’s (normal platelet morphology ,
defect of platelet aggregation )
Bernard sloer ( qualitative and mild quantitative platelet disorder , failure of platelet adhesion)
Von willebrand disease ( quantitative or qualitative deficiency of vWF molecule which - binds to exposed subendothelial collagen - mediates initial platelet adhesion
B-Acquired : Aspirin Renal failure Heparin in high doses
Secondary Hemostatic Disorders
Coagulopathies :
A- Inherited :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
B-Acquired : Vit. K defeciency Liver disease DIC ( consumptive coagulopathy ) Anticoagulants : Oral anticoagulants – Heparin
Clinical approach in making a diagnosis• 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
History
Typical Presentations Examples of typical presentations:
•A male infant who is starting to walk and presents with a painful swollen joint after a fall has hemophilia until proven otherwise.
•An adolescent girl who presents with excessive menstrual bleeding, recurrent nosebleeds, and pallor may have von Willebrand Disease (vWD), the most common inherited bleeding disorder .
Typical Presentations cont.
•A five-year-old child who is not clinically ill but presents with moderate mucocutaneous purpura in the wake of a viral infection most likely has acute post-infectious immune thrombocytopenia (acute ITP) .
•A teenage girl with easy bruising and mild pallor presenting to a pediatrician's office with a strong family history of autoimmune disorders (eg: rheumatoid arthritis, systemic lupus erythematosus, or Hashimoto's thyroiditis) may have chronic ITP .
•A ten-day-old infant with bleeding from the umbilical stump should be evaluated for factor XIII deficiency. Intracranial hemorrhage in an infant without other risk factors should also prompt consideration of this diagnosis.
• 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
Clinical Examination
Clinical approach in making a diagnosis
Hemarthrosis Ecchymosis
Purpura Petechiae
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
Localize the haemostatic defect
Vascular causes :• First and foremost rule out infectious causes “ Meningococcemia ”• Vasculitis “ Henoch-Schonlein Purpura ’’• Hemangiomas
Vascular causes :
Clinical approach in making a diagnosis
General tests
• CBC
• BT
• PT ,PTT
• TT
Laboratory Studies
CBC • Thrombocytopenia• Anemia• Leukocytosis
PT 1- Extrinsic pathway ( tissue factor , factor 7 )2- Common pathway ( 5,10,2,fib. )
Blood Film • Confirm thrombocytopenia• Signs of TTP , HUS , DIC• Malignant cells
PTT 1 - Intrinsic pathway ( 8,9,11,12 ) 2 - Less sensitive to common pathway ( 5,10,2,fibrinogen )
Labora
toy
Studie
s
High PT , normal PTT
• Liver disease• Warfarin therapy• Vit. K def.• DIC ( early )• Factor 7 def.• Factor 7 inhibitor
Dose the patient has factor def. ?
Dose the patient has factor def. ?
Do mixing tests
Corrected
Factor def.
Not corrected
Inhibitors or lupus anticoagulant
Data Interpretation
Data Interpretation
High PTT , normal PT
• Heparin therapy• Factor 8 , 9 ,11 , 12 def.• vWD• Factor inhibitor • Lupus anticoagulant
High PTT and PT
• Common pathway def.• Heparin or warfarin overdose • Late liver disease• DIC• Factors inhibitors• Primary fibrinolysis• Dysfibrinoginemia
Primary fibrinolysis
DIC Liver disease
Platelet normal Factor 8 low Factor 8 normal
D- dimer test normal
Abnormal D - dimer
D- dimer test normal
High PTT and PT
Data Interpretation
Normal PT / Normal PTT
Consider evaluating for:• Platelet disorder • Mild factor deficiency• Factor XIII• Monoclonal
gammopathy• Abnormal fibrinolysis• a2 anti-plasmin
deficiency• Vascular disorders• Dysfibrinogenemia
Data Interpretation
Normal PT , PTT Normal PT , PTT
Think of platelet
Thrombocytopenia
Look for the cause
Normal platelet
• Do PFA-100
Think of• vWD• Factor 13 def.• Vasculopathy
Data Interpretation
Current treatment strategies
Treatment depends on the type of disorder :
Treatment IndicationPlatelet concentrate Low pt , platelet dysfunction
Fresh frozen plasma “ FFP ” Multi-factor coagulopathyFactors 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-FVIII-FIX
FVII deficiencyFVIII deficiencyFIX deficiency