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