1. The Fascinating World of Haemostasis and Thrombosis
Susan Louw

2. Disease processes
Abnormal clotting
Abnormal bleeding

5. Bleeding Disorders Vessel wall / Vascular Disorders Platelets
Coagulation factors

6. Vascular Disorders Inherited Acquired
Hereditary Haemorrhagic Telangiectasia
Connective Tissue disorders
Acquired
Simple benign bruising
Senile purpura
Scurvy
Steroid purpura

7. Hereditary Haemorrhagic Telangiectasia

8. Platelet Disorders – Q & Q:
Quantity
Failure of production (Bone Marrow failure)
Increased destruction
ITP
Drug related
Infections
TTP
DIC
Splenomegaly

9. Idiopathic Thrombocytopenic Pupura (ITP)
Chronic (women 15-50yrs) and
Acute (children)
Idiopathic or secondary (HIV!, SLE, viral infections etc.)
Platelet sensitisation
with auto-antibodies
with premature
removal by spleen

10. Idiopathic Thrombocytopenic Pupura (ITP)
Clinically: Mucocutaneous bleeding

11. Idiopathic Thrombocytopenic Purpura (ITP)
Diagnosis
Plt count 5 – 50 x109 /L on FBC with increased megakaryocytes on bone marrow biopsy
Treatment
Corticosteroids
Splenectomy etc. etc.

12. Platelet Disorders – Q & Q:
Quality
Hereditary
Glanzmann’s Thrombasthenia
Bernard-Soulier syndrome
Storage pool disease
Acquired
Anti-platelet drugs
Uraemia
Myeloproliferative and –dysplasic disorders

13. Platelet Disorders – Q & Q:
Bernard-Soulier Disease:

14. Bernard-Soulier Disease:
Qualitative and Quantitative abnormality of platelets
Large platelets
Congenital deficiency
of Glycoprotein 1b
on platelet surface
Reduced platelet numbers

15. Anti-Platelet DRUGS

16. Anti-Platelet DRUGS Aspirin: cyclo-oxygenase inhibitor
Clopidogrel: blocks ADP
receptors
Abxicimab: GPIIb/IIIa inhibitors

17. Diagnosis of Platelet Disorders
FBC and Diff
BMAT
Bleeding time
Platelet aggregation studies

18. HEREDITARY Coagulation Factor defect
Haemophilia A: defective or reduced FVIII
Haemophilia B: defective or reduced FIX
Von Willebrand’s disease: defective or reduced vWF

19. HEREDITARY Coagulation Factor defect
Mode of inheritance:
Haemophilia A: Sex linked
Haemophilia B: Sex linked
Von Willebrand’s disease: Autosomal dominant

20. HEREDITARY Coagulation Factor defect
Treatment
Factor replacement – BUT then there was….

21. HEREDITARY Coagulation Factor defect
Treatment
Factor replacement
Viral inactivation
Recombinant production
DDAVP
Supportive measures
Gene therapy… watch this space

22. Thrombotic disorders Hereditary Acquired
Deficiencies of natural anticoagulants
Antithrombin / Protein C / Proten S
Acquired
Post surgery
Post delivery
Long distance travel
Immobilisation

23. Rudolf L.K. Virchow ( )

24. Virchow’s Triad

25. Thrombotic disorders

26. Warfarin and its partner
Reasons for warfarin therapy
Many !!!!
Prevent clot formation
Prothrombotic state
Inherited or Acquired
Life saving
Side-effect: Bleeding

27. The Other Partner
Heparin