BLEEDINGGatmaitan, Raymond VincentGolpeo, Kirsten C.

Case & ObjectivesA 2 year old boy was brought for check up due to multiple hematoma over both legs.1. Discuss bleeding tendencies based on their usual

presentation2. Discuss bleeding tendencies based on the

following parameter:• PT, PTT• Platelet• BT, CT

3. Discuss and correlate normal hemostasis with the laboratory findings

HEMOSTASIS – an active process that clots blood in areas of blood vessel injury, yet simultaneously limits the clot size only to the areas of injury.

1. Primary Hemostasis – process of platelet plug formation at site of injury. Occurs within seconds of injury.

Stops blood loss from the capillaries, small arterioles and venules

3 Critical events for effective primary hemostasis1. Platelet adhesion2. Granule release3. Platelet aggregation

2. Secondary Hemostasis – Reactions of the plasma coagulation system resulting to fibrin formation. Occurs within several minutes for completion. Important in larger vessels.

Coagulation Cascade

Presentation of Bleeding DisordersIn physical examination, it should focus on whether bleeding

symptoms are primarily associated with mucous membranes or skin or the muscles and joints.

Mucocutaneous bleeding (epistaxis, menorrhagia, hematuria, GI bleeding), petechiae on the skin and mucous membranes, and small ecchymotic lesions of the skin.

DEFECTS in PLATELET or BLOOD VESSEL WALL INTERACTION

Deep Bleeding into muscles and joints with much more extensive ecchymoses and hematoma formation. Hamarthrosis (Hemophilia A).

CLOTTING FACTOR DEFECIENCY

Some individuals with mild defects or deficiencies may have no abnormal findings on PE. Also, individuals with collagen matrix and vessel wall may have loose joints and lax skin associated with easy bruising (Ehlers-Danlos Syndrome)

Von Willebrand Disease (VWD) (platelet-type vWD)Autosomal dominant

Gain of function of platelet GPIb-IX-V complex – resembles vWD type 2B (adhesion disorder)

Epistaxis, ecchymoses, menorrhagia, gingival hemorrhage

Ecchymoses

Petichaie

Hamarthrosis

Laboratory Parameterso Platelet Count – Thrombocytopenia is the

most common acquired cause of a bleeding disorders in children. Normal levels: 150-400 X 109/L

o Bleeding Time – test for adequacy of primary hemostasis or platelet function.

o Prothrombin Time – assess the clotting ability of blood and assess the extrinsic and common pathway.

o Partial Thromboplastin Time – assess the coagulation proteins of the intrinsic system and the common pathway

PLATELET COUNT EFFECT

>100,000/uL (100,000 – 140,000)

Normal Bleeding Time

50,000- 100,000/ uL Mild Prolonged Bleeding time

<50, 000/uL Bleeding after minor trauma

<20,000/uL Spontaneous bleeding

LaboratoriesPlatelet BT/CT PT PTT

Henoch Schonlien Purpura

Normal Normal Normal Normal

Hemolytic Uremic Syndrome

Normal Normal

Thrombotic Thrombocytopenic Purpura

Decreased Decreased Normal Normal

Idiopathic (immune) Thrombocytopenic Purpura

Decreased Decreased Normal Normal

Von Willebrand’s Disease

Normal Prolonged (poor platelet quality)

Prolonged

LaboratoriesPlatelet BT/CT PT PTT

Factor VIII Deficiency

Normal Normal Normal Prolonged

Factor IX Deficiency

Normal Normal Normal Prolonged

Vitamin K deficiency

Increased Increased

Peripheral Blood SmearAbnormal RBC Morphology

Hemolytic Uremic Syndrome (+) Helmet cells, spherocytes, schistocytes, and burr cells

DIC (+) Schictocytes

TTP (+) spherocytes, schistocytes, and burr cells

Giant Platelets

Developmental HemostasisNewborn infant has a reduced levels of most procoagulants and

antcoagulants. There is more marked abnormality in the preterm infant. During gestation, there is no progressive maturation and increase of the clotting factors synthesized by the liver. The extremely premature infant will have prolonged PT and PTT as well as a marked reduction in anticoagulant proteins (protein C and S)Levels of fibrinogen, factor V, and VIII, VWF, and platelets are near-normal throughout the later stages of gestation.Protein C and S are physiologically reduced, the normal factors V and VIII are not balanced with their regulatory proteins.The net effect is that newborns (especially premature infants) are at increased risk for complications of bleeding, clotting, or both.

Normal Values30-36 weeks of Gestation

Full Term 1-5 years old

6-10 years old

PT(sec) 130 (10.6-16.2)

13.0(10.1-15.9)

11(10.6-11.4)

11.1(10.1-12.0)

PTT(sec) 53.6(27.5-79.4)

42.9(31.3-54.3)

30(24-36) 31(26-36)

BT(min) 6(2.5-10) 7(2.5-13)

Platelet 150-450 X 109/L

150-450 X 109/L

150-450 X 109/L

150-450 X 109/L

Correlation of Normal Hemostasis to Lab

findingsRVIN…. Help…

Thank you!