Diagnosis & Tata Laksana Perdarahan IDAI Online Symp 22012014

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Clinical approachof bleeding problems in childhood

Novie Amelia Chozie


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Introduction

Bleeding in childhood is one of challenging

problems in daily practice.

Clinical approach :

Primary hemostasis vs secondary hemostasis defect

Clinical setting is very important

Management depend on hemostasis defect that

cause bleeding.


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http://accessmedicine.net/search/searchAMResultImg.aspx?rootterm=hemostatic+function&rootID=11219&searchType=1


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Work-up of bleeding child

Comprehensive medical and specific bleeding

history.

Detailed family history including whether there is

consanguinity. Detailed physical examination.

Selected laboratory tests.

Textbook of hemophilia, 2ndEd. 2010


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Detailed history

Symptoms: Type of bleeding

Site/s of bleeding

Age of onset

Response to hemostatic challenge:

Post trauma/injury vs spontaneous

Minor/major surgery

Underlying medical conditions

Medications:

antiplatelet drugs (nonsteroidal anti-inflammatory drugs),

anticoagulants (warfarin, heparin, low-molecular-weight heparin),

Family history


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Clinical setting

Clinically

ill child

Otherwise

healthychild

Single

hemostaticdefect

Multiple

hemostaticdefects

Acquired

Hereditary

Acquired



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Type of bleeding

Mucocutaneousbleeding ?

Deep bleeding ?

Primary hemostasis defect

Secondary hemostasis defect


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Finding Disorders ofCoagulation

Disorders of Platelets orVessel

Petechiae Rare Characteristic

Deep dissecting

hematoma

Characteristic Rare

Superficial ecchymoses Common, usually large &

solitary

Characteristic, usually small

& multiple

Hemarthrosis Characteristic Rare

Delayed bleeding Common Rare

Bleeding from superficialcuts & scratches

Minimal Persistent, often profuse

Sex of patient 80-90% male Relatively more common in

females

Positive family history Common Rare

Wintrobes Clinical Hematology, 11th ed, 2004

Clinical distinctions


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Bruises/hematoma

Age & size

Distribution

Specific form/pattern


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Bruises in child abuse

There is significant bruising or bleeding with no history

of trauma

History inconsistent with the severity of the injury

Bruising in a recognisable pattern such as a belt or hand

http://www.maine.govhttp://www.laboratoryconsultationservices.com/lcs1/index.php?option=com_content&view=article&id=47&catid=901&Itemid=1&limitstart=3


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Petechial hemorrhages

Associated with thrombocytopenia

Others :

Extensive cough

Vomiting

Strangulation


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Palpable purpura (vasculitis)


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Initial screening tests

Complete blood count (CBC) & blood smear

Assessments of platelet function:

Bleeding time: prolonged with impaired platelet function, plateletcounts reduced below 80,000100,000/mm3 or impaired vascular

integrity.

Platelet function analyzer (PFA 100)

Coagulation screening :

PT (extrinsic pathway)

APTT (intrinsic pathway)


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Pseudothrombocytopenia

Platelet clumping Platelet satellitism

Desk Reference for Hematology, 2nd

ed, 2008

PDQ Hematology, 2002


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THROMBOCYTOPENIA


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Desk Reference for Hematology, 2nd ed, 2008

PDQ Hematology, 2002

Platelet Count versus Risk of Hemorrhage


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Assess peripheral blood smear

True thrombocytopenia

Normal platelet

morphology

ITP Hereditary

thrombocytopenia

Drug

induced

Abnormal platelet :

giant/micro thrombocyte

Hereditary

thrombocytopenia

Investigating isolated thrombocytopenia

in an otherwise healthy child

Lancet 2000;355(9214):1531-1539, modified

Exclude

pseudothrombocytopenia


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Diffential diagnosis of thrombocytopenia

in patient with systemic illness

Initial evaluation of thrombocytopenia

Dilutional Increased peripheral destruction Sequestration

Massive

transfusionSplenomegaly

Infection/

SepsisDIC Platelet Ab related to

autoimmune disease,

collagen vascular disease,

idiopathicTTP/HUS

Cecil textbook of medicine, 21st ed, Philadelphia, 1999, modified


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Causes of thrombocytopenia by age & frequency

Period Common Uncommon

Neonatal Sepsis Congenital amegakaryocytic

thrombocytopenia

Asphyxia/RDS Thrombocytopenia absent radii syndrome

Alloimmune thrombocytopenia Wiskott-Aldrich syndrome

NEC Bernard-Soulier syndrome

Maternal ITP Congenital leukemia

Childhood ITP Type 2 VWD

Infection ImmunodeficienciesDrug induced thrombocytopenia Autoimmune disease : SLE, JRA

Leukemia/other marrow involvement

TTP-HUS

Pediatr Clin N Am 51 (2004) 11091140


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COAGULATION

ABNORMALITIES


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Coagulation screening tests

Beck N. Diagnostic hematology. 2009


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Etiology of bleeding based on APTT


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Initial screening investigations :

Full blood count

Baseline coagulation screen : PT, APTT, TCT, fibrinogen

Further investigation : specific factor assay, D-dimer, etc

Lankowszky, 2008


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Hereditary bleeding disorder

The most common disorder :

Hemophilia A or B

Von Willebrand Disease

Inherited platelet function defects

Less common :

Rare coagulation factor deficiencies, e.g., FXI, FVII, FXIII, or

fibrinogen.



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Defect Bleeding

manifestation

PT PTT BT Treatment

Fibrinogen variable N/ Cryoprecipitate

Prothrombin variable N PCC

Factor V Mild-moderate FFP

Factor VII Moderate-severe N N rF.VIIa

Factor VIII variable N N F.VIII or DDAVP(mild)

Factor IX variable N N F.IXFactor X variable N Plasma/PCC

Factor XI variable N N Plasma/PCC

Factor XII Non-bleeding N N No need

Factor XIII severe N N N cryoprecipitate

Clinical manifestation & treatment

Colman RW, et al. Hemostasis & thrombosis : basic rinciples & clinical practice. 2006


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Acquired coagulation disorders

Lankowszky, 2008


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Possible errors affecting the APTT results

Sample variables

Error in blood sampling (e.g. underfilling, heparin)

Contamination of tissue fluids

Insufficient mixing of blood and citrate

Wrong tubes

Errors in transport and storage

High packed cell volume

Hemolysis or activation of sample

Reagent variables Insufficient mixing of reagent

Poor storage of reagents

Expiry date of reagents


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MANAGEMENT


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Initial urgent measures

Active bleeding at presentation :

Estimate the volume of blood loss is important :

Hb level is not informative

Signs of tachycardia and/or hypotension : need for fluid replacement

If any suspicion of specific coagulation defect : Avoid puncture vein and/or artery in :

the neck hematoma compression of airway

groin areas hematoma compression of femoral nerve

Take initial blood samples : Hemostasis screening

Blood grouping and cross-matching

Platelets in hematologic & cardiovascular disorders. Cambridge, 2008


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Specific management

Further diagnostic tests :

Inhibitor screening test : mixing studies

Specific coagulation factor assay

Tests for DIC

Treatment

Treatment of underlying disease

Replacement therapy of defective hemostatic factor(s) :

Platelet transfusion FFP

Cryoprecipitate

Coagulation factor concentrate


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Platelet Transfusions in Children Indicated

Platelet count


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FFP transfusion

FFP contains normal adult concentrations of bothprocoagulant and anticoagulant hemostatic factors (1

unit/ml).

Indication : To restore hemostatic levels of coagulation proteins resulting frommultiple factor deficiency.

To provide hemostatic levels of specific coagulation factors, such

as factor V, that are not available as pharmaceutical concentrates.

INAPPROPRIATE for volume replacement or as albumin

source.

Dosage : 10 20 ml/kgBW

Handbook of Pediatric Transfusion Medicine, 2004


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Cryoprecipitate transfusion

Cryoprecipitate contains : Fibrinogen (180 250 unit per 15 ml)

F VIII (80 100 unit per bag)

F Von Willebrand

F XIII

Indications : Fibrinogen deficiency

F XIII deficiency

Hemophilia A*

Von Willebrand disease*

Dosage : 5 10 ml/kgBW



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Coagulation factor concentrates

F VIII concentrates : plasma derived and recombinant

F IX concentrate (plasma derived)

Prothrombin Complex Concentrates (F II, VII, IX, X)

Factor VIIa (recombinant)



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Pearls

Clinical history provides the most useful information.

The physical examination should focus on whether

symptoms are primarily associated with mucocutaneous

bleeding or deep bleeding.

Nowhere in laboratory medicine is getting a good

specimen more important than in coagulation.


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Recognition of bleeding

as involving primary or secondary hemostasis

is criticalin organizing the diagnostic

and therapeutic approach

to bleeding disorders.


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THANK YOU

Diagnosis & Tata Laksana Perdarahan IDAI Online Symp 22012014

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