BLEEDING

1. BLEEDING Gatmaitan, Raymond Vincent Golpeo, Kirsten C.

2. Case & Objectives A 2 year old boy was brought for check up due to multiple hematoma over both legs. • Discuss bleeding tendencies based on their usual presentation • Discuss bleeding tendencies based on the following parameter: • PT, PTT • Platelet • BT, CT • Discuss and correlate normal hemostasis with the laboratory findings

3. 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. • 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 hemostasis • Platelet adhesion • Granule release • Platelet aggregation • Secondary Hemostasis – Reactions of the plasma coagulation system resulting to fibrin formation. Occurs within several minutes for completion. Important in larger vessels.

7. Coagulation Cascade

8. Presentation of Bleeding Disorders In 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)

9. Von Willebrand Disease (VWD) (platelet-type vWD) Autosomaldominant Gain of function of platelet GPIb-IX-V complex – resembles vWD type 2B (adhesion disorder) Epistaxis, ecchymoses, menorrhagia, gingivalhemorrhage

10. Ecchymoses

11. Petichaie

12. Hamarthrosis

13. Laboratory Parameters • Platelet Count – Thrombocytopenia is the most common acquired cause of a bleeding disorders in children. Normal levels: 150-400 X 109/L • Bleeding Time – test for adequacy of primary hemostasis or platelet function. • Prothrombin Time – assess the clotting ability of blood and assess the extrinsic and common pathway. • Partial Thromboplastin Time – assess the coagulation proteins of the intrinsic system and the common pathway

15. Laboratories

16. Laboratories

17. Peripheral Blood Smear

19. Giant Platelets

20. Developmental Hemostasis Newborn 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.

21. Normal Values

23. Correlation of Normal Hemostasis to Lab findings • RVIN…. Help…

24. Thank you!