How To Approach A

child with Bleeding

Disorder

DR. KAMRAN AKBAR

PGR PEDIATRICS

UNIT 2

Approach includes:

History

Clinical Examination

Laboratory Findings

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

HISTORY:

If child bleeds

from superficial

cut with profuse

bleed?

If fever and

neurological

manifestations?

If history of viral

prodrome ?

Drug related

purpuraMeningococce

mia/TTP

If patient taking

aspirin ,

ibuprofen

,antibiotics.

Coagulation

disorderPlatelet Disorder HUS/ITP

If child bleeds

from injury ,

does the

bleeding stop

and resume?

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.

DICVitamin K

DeficiencyCongenital Disorder

Post transfusional

purpura or

alloimunizational

HISTORY: continued

Clinical Examination

The examination should determine the presence of :

petechiae,

ecchymosis,

hematomas,

hemarthroses,

or mucous membrane bleeding.

Petechiae Purpura

Senile Purpura DIC

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)

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

Bleeding Disorders

Vessel Wall

DisorderPlatelet Disorder

Coagulation

Factor

Abnormalities

Vessel wall disorder

Acquired Congenital

1. SENILE PURPURA

2. VASCULAR PURPURA

3. HENOCH SCHONLEIN PURPURA

1. HEREDITARY HEMORRHAGIC

TELENGIECTASIA

2. EHLERS DANLOS SYNDROME

Henoch-schonlein purpuraEhlers-Danlos syndrome

HEREDITARY HEMORRHAGIC

TELENGIECTASIA

HEMOPHILIA

Platelet Disorders

Qualitative Quantitative

• THROMBASTHENIA

• BERNARD-SOULIER SYNDROME

• DRUGS(ASPIRIN,TXA2,INDOMATHACIN

• THROMBOCYTOPENIA

• THROMBOCYTHEMIA

Coagulation

Factor

Abnormalities

Hereditary Acquired

1. HEMOPHILIA A(factor VIII deficiency)2. HEMOPHILIA B(factor IX deficiency)3. von WILLEBRAND DISEASE4. DISORDERS OF FIBRINOGEN-

HEREDITARY AFIBRINOGENAEMIA HYPOFIBRINOGENAEMI DYSFIBRINOGENAEMIA

1. DISSEMINATED INTRAVASCULAR COAGULATION(DIC)2. LIVER DISEASE3. VIT K DEFICIENCY4. MASSIVE TRANSFUSION OF STORED BLOOD5. ACQUIRED INHIBITORS OF COAGULATION6. HEPARIN OR ORAL ANTICOAGULANT THERAPY7. RENAL DISEASE

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

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

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

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)

Bleeding disorder

Von willebrand

disease (vWD)

Treatment

Desmopressin acetate (DDAVP)

Treatment of common bleeding

disorders (continued)