1. Bleeding Disorders Dr. Farjah H.AlGahtani
Assistant Professor of Internal Medicin,Consultant Hematology
Director of transfusin Medicin and Blood Bank Department,KSU

2. Stable Hemostatic Plug
Hemostasis
CBC-Plt
BT,(CT) PT
PTT
BV Injury
Neural
Damage/contact.
Contact
Coagulation
Cascade
Blood Vessel
Constriction
Platelet
Aggregation
Primary hemostatic plug
Reduced
Blood flow
Platelet
Activation
Fibrin formation
Platelet study
Antibody tests
Factor Assay
Stable Hemostatic Plug

3. HEMOSTASIS Primary Hemostasis Secondary Hemostasis Tertiary Hemostasis
Blood vessel contraction
Platelet Plug Formation
Secondary Hemostasis
Activation of Clotting Cascade
Deposition & Stabilization of Fibrin
Tertiary Hemostasis
Dissolution of Fibrin Clot
Dependent on Plasminogen Activation

4. Classification: Disorders of Blood vessels Disorders of Platelets
Scurvy, senile purpura, Henoch-Schonlein syndrome.
Disorders of Platelets
Thrombocytopenia ITP, TTP, HUS, DIC.
Aspirin therapy, Thrombasthenia,
Disorders of Coagulation
Extrinsic, intrinsic, combined.
Other disorders
Post transfusion purpura, MPS, MDS.

5. Tests of Hemostasis: Screening tests: Specific tests:
Bleeding.T - 10m. Platelet & BV function
Prothrombin.T – Extrinsic, aPTT – Instrinsic
Thrombin.T – common path. (DIC)
Specific tests:
Factor assays – hemophilia.
Tests of thrombosis – TT, FDP, DDA,
Platelet function studies:
Adhesion, Aggregation, Release tests.
Bone Marrow study

6. Bleeding: Clinical Features
Local - Vs - General, spontaneous . .
Hematoma / Joint Bleeds- Coag
Skin / Mucosal Bleeds – PLT
wound / surgical bleeding –
Immediate - PLT
Delayed - Coagulation

7. Platelet Coagulation
Petechiae, Purpura Hematoma, Joint bl.

8. Vascular disorders:
Petechiae, purpura, ecchymoses
senile purpura
vitamin C deficiency (scurvy)
Connective tissue disorders
Infections – Meningococcus
Henoch-Schonlein Purpura-Immu

9. Senile Purpura

10. (Rocky Mountain Spotted Fever)
Petechiae in
Vasculitis
(Rocky Mountain Spotted Fever)

11. Henoch-Schonlein purpura
Immune disorder
Children
Follows infection
Petechiae with edema and itching.

12. Henoch-Schonlein purpura
20y Male, fever, painful symmetric polyarthritis for a day. During the next two days, edema and palpable purpura developed.

13. Platelet Disorders - Features:
Mucocutaneous bleeding
Petechiae, Purpura, Ecchymosis.
spontaneous bleeding after trauma
CNS bleeding (severe  plt)
Prolonged bleeding time (BT)

14. BLEEDING TIME vs. PLATELET COUNT

16. Idiopathic Thrombocytopenic Purpura (ITP)
Acute - children (post infection)
Chronic - adults ( females, yrs)
autoimmune disorder
antiplatelet antibodies (IgG)
IgG coated platelets removed by spleen
Usually  megakaryocytes in BM

17. Platelet dysfunction:
Inherited Disorders:
Bernard-Soulier disease
large platelets, failure of adhesion
Glanzmann’s thrombasthenia
normal size, failure of aggregation
Acquired Disorders:
Drugs - Aspirin, Alcohol, Uremia,

18. Platelet Aggregation Curves

19. Von-Willebrand Disease:
Coagulation + PLT disorder:
Congenital disorder
Deficiency of vWF molecule
Part of FVIII,
Mediates platelet adhesion
Prolonged Bleeding time
Low Factor VIII & long aPTT
Mucocutaneous bleeding

20. Von-Willebrand Disease
vWF: F-VIII & PLT function.
Defective Platelet Adhesion
Skin Bleeding
Prolonged Bleeding time.
Low Factor VIII levels.

21. Coagulation disorders:
Deficiencies of Clotting factors
Onset - delayed after trauma
Deep bleeding
Into joints - Hemarthroses
Into deep tissues – Hematoma
large skin bleed – Ecchymoses

22. Blood Coagulation & Tests

23. CT- Large hematoma of psoas muscle

24. Coagulation Disorders
Laboratory findings:
Normal bleeding time & Platelet count
Prolonged prothrombin time (PT)
deficiencies of II, V, VII, X
Prolonged time (aPTT)
all factors except VII, XIII
Mixing studies - normal plasma corrects PT or aPTT

25. Factor VIII Deficiency
Classic hemophilia (hemophilia A)
X-linked disorder (affects 1º males)
Most common - severe bleeding
Spontaneous hematomas < 1, 5, 75%
Abnormal aPTT – Intrinsic path.
Diagnosis - factor VIII assay
Treatment - factor VIII concentrate
Cryoprecipitate (less desirable)

26. Factor IX Deficiency Christmas disease (Hemophilia B)
X-linked recessive disorder
Indistinguishable from classic hemophilia (F VIII)
Requires evaluation of factor VIII and IX activity levels to diagnose
Treatment - factor IX concentrate
Cryoprecipitate if factor IX unavailable

27. Secondary Hemostatic Disorders
Acquired coagulation disorder:
Vitamin K deficiency
- neonates - decreased intestinal
flora and dietary intake
- oral anticoagulants (coumadin)
- fat malabsorption syndromes
Required for factors II, VII, IX, X
Prolonged PT and aPTT

28. Combined Primary and Secondary Hemostatic Disorders (DIC)
Disseminated Intravascular Coagulation
Major pathologic processes -
obstetric complications, neoplasms, infection (sepsis), major trauma
Primary - platelet consumption
( bleeding time,  platelets)
Secondary - factor consumption
( PT, aPTT)

29. Combined Primary and Secondary Hemostatic Disorders
Severe Liver Disease
Primary - dysfunctional platelets and/or thrombocytopenia ( BT)
Secondary - decrease in all coagulation factors except vWF ( PT, aPTT)
Vitamin K will promote synthesis of factors II, VII, IX, X

30. Summary Hemostatic Disorders
BT Plt PT PTT
Vascular Dis - 
PLT Disorder -  - 
Factor 8/9
*Congenital 
Vit K / Liver
*Acquired  -
Combined (DIC)   - 

31. Summary

32. CLL-Thrombocytopenia
Lymphoid cells
Smear cells
(Fragile)

33. Dengue Hemorrhagic fever
Platelet deficiency..

34. Thrombocytopenia-TTP

35. Summary Complex system to keep blood fluid To block leakage on injury.
BV, PLT & Coagulation
Complex inhibitory mechanisms
Complex thrombolysis mechanisms.
Screening tests: BT, CT (PT, aPTT)
Special tests: Factor assay, PLT function etc.

36. Disorders of Hemostasis
Vascular disorders –
Scurvy, easy bruising, Henoch-Schonlein purpura.
Platelet disorders
Quantitative - Thrombocytopenia
Qualitative - Platelet function disorders – Glanzmans
Coagulation disorders
Congenital - Haemophilia (A, B), Von-Willebrands
Acquired - Vitamin-K deficiency, Liver disease
Mixed/Consumption: DIC