Hemorrhagic disorders involve problems with hemostasis, the process of stopping bleeding. There are bleeding disorders which can be caused by platelet defects or vascular defects. Hemorrhagic disorders involve deficiencies in coagulation factors. Common hemorrhagic disorders include hemophilia A which is a factor VIII deficiency and hemophilia B which is a factor IX deficiency. Both hemophilia A and B are inherited disorders that primarily affect males. Treatment involves replacement of the deficient coagulation factor through products such as fresh frozen plasma or concentrated factor products.

1. Hemorrhagic disorders
Micheal Mohab Nady Mikael

2. Hemorrhagic disorders
Hemostatic system
Hemostasis is the dynamic process of coagulation .It stop
bleeding in the areas of vascular injury .This process
involves the interaction of
-cellular component:
- Platelets
- Vascular wall
- Protein component :
- Procoagulants( Coagulation factors )
-Fibrinolytics ( for lysis)
-Anticoagulants (regulation)

3. Blood vessel injury →vasoconstriction &
exposed endothelium & sub endothelial
collagen →stimulation of von Willebrond
factor→ platelets activation & adhesion
→activated platelets induce platelets
aggregation .At the same time tissue factor
+ collagen &other proteins in the tissue
activate the coagulation cascade
→formation of thrombin which has
multiple effects on coagulation mechanism :

4. -feedback activation of factors 5&8
-conversion of fibrinogen to fibrin
-activation of factor 2thus a platelet
plug is formed &bleeding stop .If
failed then thrombin activate
-factor 13 which links fibrin to form
a stable thrombus. Then contractile
elements within platelets cause clot
retraction

5. • Thrombin also activate the anticoagulant
system leading to limitation of clot size
.Thrombin activate
• the fibrinolytic system to cause lyses of the
thrombus resulting in recanalysation of the
blood vessel.

6. Normal hemostasis
• Components
First limb:
• Coagulation factors
second limb:
Cell component (platelets and endothelial cells)
• Rgulation
– Coagulation system
– Fibrinolysis system
– Anticoagulation system

7. Hemostasis
BV Injury
Platelet
Aggregation
Platelet
Activation
Blood Vessel
Constriction
Coagulation
Cascade
Stable Hemostatic Plug
Fibrin
formation
Reduced
Blood flow
Tissue
Factor
Primary hemostatic plug
Neural
Lab Tests
•CBC-Plt
•BT,(CT)
•PT
•PTT
Plt Study
Morphology
Function
Antibody

8. Kinins HMW Kininogen
Kallikrein Contact Activation
XII Prekallikrein
XIIa
XIa XI
IXa Ca++ IX
VIIIa VIII
Ca++
Phospholipid
Intrinsic Pathway
Xa X
Va V
Ca++
Phospholipid XIII
II IIa
XIIa
Fibrinogen Fibrin XIII
Common Pathway
VII VIIa
Ca++
Tissue Factor
Coagulation cascade
Extrinsic Pathway

12. Platelet structure:
Platelets are non-nucleated cellular
fragments produced by megakaryocytes of
bone marrow. Their life span is 7-10 days.
Normal platelet count is 150 - 400 x 109/ L.
(150.000-400.000/mm3).

13. Functions of platelets
a- They stimulate vasoconstriction of the injured vessels.
b- They form a primary hemostatic plug to seal small
vascular tears. This plug is formed by platelet adhesion
(sticking of the platelets to the subendothelial structures)
followed by aggregation (sticking of the platelets to each
other).
c- They play an essential role in fibrin clot formation by
the production of platelet factor 3 needed in blood clot
formation.
d- They induce fibrin clot retraction.

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

15. Hemostatic Disorders
1-bleeding disorders
a-platelets defects
b-vascular defects
2-Hemohrragic disorders

16. 1-bleeding disorders
Vascular defect -  fragility
Petechiae, purpura, ecchymoses
 senile purpura
 vitamin C deficiency (scurvy)
 connective tissue disorders

17. Vascular defect - cont.
 Infectious and hypersensitivity
vasculitides
- Rickettsial and meningococcal
infections
- Henoch-Schonlein purpura
(immune)

18. Platelet disorders:
  platelets (thrombocytopenia)
petechiae
spontaneous bleeding after trauma
CNS bleeding (severe  plt)
 Platelet dysfunction -
mucocutaneous bleeding

19. 2-Hemohrragic disorders
• Coagulation factors defect
• Vitamin K deficiency
• Anticoagulant therapy: heparin

20. Bleeding Time Test
Timer is started upon
incision
Bleeding time = time
to complete cessation
of free blood flow from
incision

21. Tests for coagulation factors
• Clotting time
• Thrombin time
• Prothrombin time
• Partial thromboplastin time

22. purpura
Classification of purpuras
A- Purpura associated with normal numbers of
platelets (non thrombocytopenic purpuras)
1- Vascular purpura
a- Henoch-Schonlein purpura (anaphylactoid
purpura).
b- Meningococcemia or septicemia.
c- Toxic vasculitis: may produce hemorrhagic rash
as a reaction to drugs such as iodides.
d- Viral or rickettsial infections.
e- Scurvy (vitamin C deficiency).

23. 2- Platelet function disorders
a- Congenital platelet function defects
(thrombasthenias).
b- Acquired platelet function disorders: e.g.
uremia.
c- Drug-induced abnormalities of platelet
aggregation (salicylates therapy).

24. B- Purpura associated with decreased number of
platelet (thrombocytopenic purpuras)
1- Idiopathic thrombocytopenic purpura (ITP).
2- Aplastic anemia.
3- Bone marrow infiltration e.g. leukemia.
4- hypersplenism.
5- Other thrombocytopenic purpura.
a- Drug induced thrombocytopenia e.g
carbamazepine.
b- Congenital thrombocytopenic syndromes (e.g.
Wiskott Aldrich syndrome).
c- Thrombocytopenia with cavernous hemangioma.
6- Neonatal thrombocytopenia.

25. Idiopathic Thrombocytopenic
Purpura (ITP)
 Acute - children (post infection)
- autoimmune disorder
- antiplatelet antibodies (IgG
against platelet
glycoproteins)
- IgG coated platelets removed by
spleen ( platelet survival)
 Usually  megakaryocytes in BM

26. Clinical Picture
 The classic presentation of ITP is at age 1-
4 years.
 Sudden onset of generalized petechial
purpura in a previously healthy child.
 Intracranial hemorrhage
 no organomegally
 70-80% spontaneous recovery.
 About 10-20% develop chronic ITP
persisting more than 6 months.

27. Laboratory investigations:
 Platelet count is reduced below 150x109
/L, bleeding occurs with platelet count<40
x109/L.
 Bleeding time and clot retraction test yield
abnormal results.
 Anemia is not present unless significant
blood loss has occurred.
 White blood cell count is normal.

28.  Bone marrow aspiration reveals
normal granulocytic and erythrocytic
series, normal or increased numbers of
megakaryocytes, some of
megakaryocytes are immature, platelet
budding may be scanty but there is no
diagnostic megakaryocyte morphology.

29. D.D:
Non thrompocytopenic purpura:
Other thrompocytopenic purpura:
 Aplastic anemia
 Bone marrow infiltration
 hypersplenism

30. Treatment
• Fresh blood
• PLT transfusion
• IV immunoglobulin
• Steroid therapy
• IV antiD therapy
• splenectomy

31. HENOCH-SCHÖNLEIN PURPURA

32. DEFINITION
• Also called “anaphylactoid purpura”
• HSP is a systemic vasculitic syndrome
with:
– Palpable purpura
– Arthralgias or arthritis
– GIT involvement
– Glomerulonephritis

33. EPIDEMIOLOGY
• 90% of cases reported in children
– Peak in children aged 4-7
• Male:Female (1.5:1)
• 50% follow a URI
• Renal disease is more severe in adults

34. CLINICAL FEATURES
• Tetrad of symptoms
– Abdominal pain
– Renal disease
– Palpable purpura
– Arthritis/arthralgias – more common in adults
and most common in knees and ankles.
Generally self-limiting

35. MANAGEMENT
• Usually self-limiting (1-6 weeks)
• Steroids:
– may decrease tissue edema, may aid in
arthralgias and some abdominal pain
– Has not been shown to be beneficial in kidney
disease or dermal manifestations
– Does not lessen chance of recurrence
– Does not shorten duration of disease

36. Coagulation disorders
Heridetary:
- hemophilia A
- hemophilia B
- hemophilia C
Acquired:
• Hepatic dysfunction
• Vit K defeciency
• DIC

37. Hemophilia

38. Hemophilia
• Caused by an absence or decreased
amount of a procoagulant –
• VIII -Hemophilia A affects ~ 1:5000
males
• XI -Hemophilia B affects ~ 1:30000
males
• XI –Hemophilia C – Rare /Ethnicity

39. Epidemiology
Hemophilia
A
Other
Hemophilia
B
Incidence: Hemophilia A - 1:5,000
Hempohilia B – 1: 30, 000

40. Inheritance

41. Inheritance

42. Woman can have hemophilia
• Lyonization of the normal X
chromosome
• Turner syndrome ( XO)
• Father with hemophilia/ mom as a
carrier
• vW type 2 N ( Normandy)

43. Clinical Severity

46. Laboratory findings:
• Rolonged PTT
• Normal PT and thrombin time
• Decreased level of factor VIII

47. Complications :
• Joint derormities
• Inhibitors to factor VIII
• Hepatitis
• AIDS

48. management
• General: dental hygiene, avoid
injury,avoid aspirin,replacement therapy
with surgery
• Replacement therapy:
1. Fresh frozen plasma
2. Factor VIII concentrates
3. Intranasal form of desmopressin acetate

49. prophylaxis
• Factor VIII concentrates (20-40IU/Kg)
every 3 days with severe form

50. Thank you