Bleeding Disorder Hemostasis Thrombocytopenia, ITP.ppt

HEMOSATSIS

1. Bleeding Disorders
Dr M H Shariff

2. Learning Objectives
Normal Hemostasis
Classification of Hemostatic Disorders
Thrombocytopenia
ITP

3. HEMOSTASIS

4. Major Components of
normal hemostasis
Vascular
Platelets
Coagulation

9. Coagulation Factors
Factor I (Fibrinogen)
Factor II (Prothrombin)
Factor III (Thromboplastin)
Factor IV (Calcium)
Factor V (Proaccelerin)
Factor VII (Proconvertin)
Factor VIII (Antihemophilic A)
Factor IX (Christmas)
Factor X (Stuart prower)
Factor XI (PTA)
Factor XII (Hageman factor)
Factor XIII (Fibrin Stabilizing)

11. Coagulation –inhibitory system
Limit clotting to the vicinity of tissue
injury

12. Inhibitors Plasma Conc
(mg / ml)
Throm
bosis
Coagulation
Factor
Serine protease Inhibitors
Anti thrombin III1-1.15 x 10
-1
YesXa, Thrombin
Heparin cofactor II ?Thrombin

2Macroglobin 2.5 ?Thrombin
Kallikrien
Protein C System
Protein C 4 x 10
-3
YesVa, VIIIa
Protein S 3 -4 x 10
-2
YesCofactor–Protein C

13. Fibrinolytic system
Physiological function of
Fibrinolytic enzymatic system is to digest
intravascular deposits of fibrin
(thrombi) in both large and small vessels &
extravascular fibrin of haemostatic plugs
and inflammation

18. CLASSIFICATION OF
HEMOSTATIC DISORDERS

19. Major Components of
normal hemostasis
Vascular Defect
Platelets Decfect –Quantitative
-Qualitative
Coagulation Defect –Congenital
-Acquired

20. Vascular
Congenital
Acquired
Hereditary hemorrhagic telangiectasis
Ehlers danlos disease
Simple bruising
Senile purpura
Vascular Purpuras
Miscellaneous
Infections, dysproteinemias
Drugs, Cushing’s disease
Uraemia, scurvy
Henoch –Schoelein syndrome
Fat embolism
Auto erythrocyte sensitization
Mechanical purpura
Orthostatic purpura
Systemic disorders

21. Coagulation Disorders
Congenital
Acquired

22. Platelet
Quantitative
Qualitative

23. Qualitative disorders
Congenital
Membrane receptor defect
Enzyme defect
Granule defect
Acquired
Stem cell disorders
Drugs
Dysproteinemias
Uremia
Miscellaneous
Glanzmans thrombosthenia
Bernard soulier syndrome
Pseudo von willebrand disease
Phospholipase deficiency
Cyclo oxygenase deficiency
Thromboxane synthatase deficiency
Storage pool deficiency
Gray platelet syndrome

24. Thrombocytopenia
Classification
 Acquired
 Congenital

25. Acquired
Idiopathic thrombocytopenic purpura
Bone marrow infiltration
Aplastic anemia
Leukemias
DLE
Hypersplenism
Infections
Megaloblastic anemias
Blood transfusion
Alcoholism
Liver disease
DIC
Post partum thrombocytopenia
Post transfusion thrombocytopenia
Thrombotic thrombocytopenia
Hemangioma
Food allergy
Idiopathic cryoglobulinemia

26. Congenital
Immune
Infections
Drugs
Congenital leukemia
Congenital megakaryocytic Hypoplasia
Giant hemangioma
Hereditary

27. Idiopathic Thrombocytopenic
Purpura
Primary Thrombocytopenic Purpura
Immune Thrombocytopenic Purpura
Purpura hemorraghica
Werlhof’sdisease

28. Clinical features
Age -children & young adults
Sex-females 3:1
Presents–bleeding Spontaneous
Trauma
Surgery
Dental procedures
Site of bleeding Skin
Mucous membrane
Nose
Hematuria
Menorrhagia
Metrorhagia
Malaena

32. Blood picture
Bleeding time -prolonged
Tourniquet test –positive
Peripheral smear
Decreased platelets
Anemia

33. Bone marrow
Erythroid series
Myeloid series
megakaryocytic series

35. Diagnosis
Thrombocytopenia
Megakaryocytes –normal / increased
Anti platelet antibodies

36. HOW DO YOU INVESTIGATE
A CASE OF BLEEDING
DISORDER?

37. Screening tests
Platelet count
Platelet morphology
Skin bleeding time
Diagnostic
Platelet aggregometry
Laboratory Investigations

38. Screening tests
Prothrombin time (PT)
Activated Partial Thromboplastin time (APTT) and INR
Thrombin clotting time (TCT)
Euglobin Lysis time
Clot solubility test
Fibrin degradation products
Diagnostic
Factor assay
DNA probe