DR. KHADIJA HABIB PG TRAINEE (MCPS)

PAEDIATRICS

DIAGNOSTIC APPROACH TO A PATIENT PRESENTED WITH

BLEEDING(IRRESPECTIVE OF AGE)

HEMOSTASIS

Three main components of hemostasis:1. Vascular wall2. Platelets3. Coagulation cascade

Two important functions:4. To maintain the blood in fluid clot free state in

blood vessels5. To induce rapid and localized hemostatic

plug at site of injury

PROCOAGULANT = TXA2

ANTICOAGULANT = PGI2 & NO

HEMOSTATIC PLUG

PRIMARY PLUG SECONDARY PLUG

PLATELETSCOAGULATION CASCADE

PRIMARY PLATELET PLUG

Platelets are responsible for primary hemostasis and carried out in 4 steps:

1. Adhesion2. Activation3. Secretion4. Aggregation

1: ADHESION

vWF

Dense granules (activation) = ADP, serotoninAlpha granules (adhesion) = PF4, PDGF, vWF, fibrinogen

GP Ib/IX/V

2: ACTIVATION

vWF

GPIa/IIa

GPIIb/IIIa

Fibrinogen

GPIIb/IIIa conformational change isInduced with epinephrine, ADP, serotonin, Arachidonic acid, thrombin

3: AGGREGATION

Collagen

vWF

GpIIb/IIa

SECONDARY HEMOSTATIC PLUG(Coagulation proteins)

BT inc , Thrombocytopenia

BT inc , Platelet count normal

1. ITP2. TTP3. HUS4. HIT

1. Aspirin 2. Uremia

Main genetic disorders3. Bernard soulier

syndrome4. Glantzmann’s

thrombasthenia5. vWF

SUPERFICIAL BLEEDING(epistaxis , petechiae , echymosis)

BT / CBC

vWF disease1. BT inc

2. Platelets normal3. PT normal

4. aPTT raised

Work up for prolonged PT

Work up for prolonged APTT

INHERITED 1. Factor VII deficiency

ACQUIRED2. Factor VII deficiency3. Vitamin K deficiency4. Liver diseases5. Warfarin administration6. Inhibitor of factor VII

INHERITED1. Deficiency of factor VIII , IX & XI2. Deficiency of factor XII ,

prekallikrein or HMW kininogen3. VWD

ACQUIRED4. Heparin administration5. Inhibitors of factor VIII , IX , XI

& XII6. Acquired VWD7. Lupus anticoagulant

DEEP BLEEDING(hematoma , hemarthrosis)

PT / APTT

MIXING STUDIES / CORRECTION STUDIES

When abnormality in PT and APTT is detected then mixing studies is performed to rule out:

1. Presence of Inhibitor to clotting factor2. True clotting factor deficiency

1: PRESENCE OF INHIBITOR TO CLOTTING FACTOR

2: TRUE CLOTTING FACTOR DEFICIENCY

Aged serum: (1 8 5 2)1. Factor I2. Factor VIII3. Factor V4. Factor II

Adsorbed plasma: (1 9 7 2)1. Factor X2. Factor IX3. Factor VII4. Factor II

TEST V VII VIII IX X XI or XII

PT Abnormal

Abnormal

Normal Normal Abnormal

Normal

APTT Abnormal

Normal Abnormal

Abnormal

Abnormal

Abnormal

ADSORBED PLASMA

Corrects No change

Corrects No change

No change

Corrects

AGED PLASMA

No change

Corrects No change

Corrects Corrects Corrects