1. HPI: A 4-year-old female. Referred to the hematology department. C/C:
easy bruising and "rash" for 3 days.
HPI:
She developed an acute onset of easy bruising and "rash" 3 days ago.
She has not had epistaxis, oral bleeding, gross blood in urine or stools.
No hemarthrosis.
No joint pain.
SCENARIO

2. HPI: Travel Hx.: Family Hx.: SCENARIO No fever. No appetite change,
No weight loss.
She had URTI symptoms approximately 2 weeks ago.
Travel Hx.:
No travel history..
Family Hx.:
She has 2 older brothers, neither of whom have had bleeding symptoms.
Family hx. is –ve for any bleeding tendency.
No hx. of malignancy or autoimmune diseases.
SCENARIO

3. Physical Examinations:
Past Hx.:
No past hx of a similar problem.
No past hx. of prolonged bleeding of wound or dental extraction.
She had an URTI 2 weeks ago.
Physical Examinations:
Vital signs:
Normal.
Growth parameters:
SCENARIO

4. Physical Examinations:
Head and Neck:
No bleeding or bruises.
Chest Examinations:
Heart & Lung are normal.
Abdominal Examinations:
No tenderness.
No organomegally.
She has diffuse petechial rash is noted on her neck, trunk, extremities and groin. It is non-blanchable or varying ages.
CNS Examination:
Normal.
SCENARIO

5. Peripheral blood smear:
Lab.:
CBC:
Hb: 12.8 g/dl.
Hct: 38.5 %.
WBC: 6,000 with normal differential.
Platelets: 5,000 “low”
PT: 12 seconds.
PTT:32 seconds.
Peripheral blood smear:
Normal RBCs & WBCs.
Platelets: reduced in number and large size.
SCENARIO

6. Dx.:
ITP
SCENARIO

7. ► Bleeding Disorders

8. Review Hemostasis

9. Primary Secondary Tertiary Hemostasis
It is the process to stop blood loss.
Three Phases:
Primary
Secondary
Tertiary

10. Primary Hemostasis

11. PRIMARY HEMOSTASIS PRIMARY HEMOSTASIS: Platelets Adhesion:
Release of vaso-constrictors
Endothelium Injury
Exposure of Subendothelial Collagen
PRIMARY HEMOSTASIS
Platelets activated and release ADP & TXA2
Platelets Adhesion:
vWF adhers Platelets to Subendothelial Collagen Via GPIb
Platelets Aggrigation
These Factors (ADP & TXA2) Stimulate Platelets to bind together via GPIIb & GPIIIa

12. PRIMARY HEMOSTASIS:
PRIMARY HEMOSTASIS

13. Ends with formation of platelets plug.
Primary Hemostasis
Ends with formation of platelets plug.
Platelet
GPIIb/GPIIIa
vWF
GPIIb/GPIIIa
Platelet
Assessment:
Platelets Counts:
150 – 450 X 103/ml.
Bleeding Time BT:
<8 mins.
GPIb
vWF

14. Secondary Hemostasis

15. SECONDARY HEMOSTASIS PT/ aPTT INR SECONDARY HEMOSTASIS: Intrinsic
Depends on the activities of coagulation factors.
Ends with formation of Fibrin clot.
Intrinsic
Extrinsic
aPTT
PT/
INR
SECONDARY HEMOSTASIS PT
Thrombin
aPTT TT

16. Secondary Hemostasis
PT: Prothrombin Time: - Normal: 11 – 24 sec. aPTT: activated Partial Thromboplastin Time: - Normal: 22 – 35 sec. INR: International Normalization Ratio: - Normal: 0.9 – 1.2

17. Secondary Hemostasis
PT: Play Tennis: - Tennis is played outside → Extrinsic Pathway. PTT: Play Table Tennis: - Table Tennis is played inside → Intrinsic Pathway.

18. Resolution

19. Fibrin Stabilization:
Resolution:
Secondary Hemostasis
Fibrin Stabilization:
Conversion from soluble to insolible clot.
Fibrinolysis:
Once healing is initiated, clot dissolution.

21. Disorders of Primary Hemostasis

22. Primary Hemostasis
Primary Hemostasis
Platelets Disorders
Vascular
vWD
Platelet
GPIIb/GPIIIa
vWF
GPIIb/GPIIIa
Platelet
GPIb
vWF
Characterized by superficial bleeding. petechiae, ecchymoses

23. Primary Hemostasis
Disorders of

24. Disorders of Primary Hemostasis
Platelets Disorders
Low Platelets Count
“Thrombocytopenia”
Platelets Dysfunction
“Thrombophillia”
↓Production
↑Destruction
Sequestration
Heridatery
Acquired
Bernard Soulier Syndrome:
“GPIb Deficiency”
Glanzmans Syndrome:
“GPIIb/IIIa Deficiency.
Drugs.
Uremia.
Aplastic anemia
ITP
TTP HUS
Splenomegaly

25. Immune Thrombocytopenic Purpura ITP

26. ITP ITP: Common cause of thrombocytopenia. Peak age: 2 – 6 years. M=F.
Pathophysiology:
Antibodies bind to platelets membranes → destruction by spleen.
ITP

27. ITP C/P: 50% presents 1-3 weeks after viral illnesses (URTIs).
Sudden onset of petechiae, purpura, echymosis, epistaxis,...
No hepatomegaly or splenomegaly.
ITP

28. ITP Lab. Findings: CBC: Platelets count: Mild: < 100,000/mm3.
Moderate: < 50,000/mm3.
Severe: < 20,000/mm3.
Bleeding Time BT: prolonged.
PT & aPTT are normal.
ITP

29. ITP Treatment: Mild cases: No treatment is required.
Severe cases: (Platelets count < 50,000/mm3 ):
Prednisolon.
IVIG.
Dangerous bleeding (e.g., intracrainial):
Platelets.
High dose steroids.
Emergency splenectomy.
ITP

30. ITP Treatment: Chronic ITP: Cytotoxic drugs: e.g., cyclophosphamide.
Splenectomy if not responding to medical treatment.
ITP

31. Henoch-Schönlein purpura (HSP)

32. Henoch-Schönlein purpura (HSP)
Also called:
Allergic Purpura, or
Anaphylactoid Purpura.
 Henoch-Schönlein purpura (HSP) 

33. ITP HSP: Etiology: Unknown.
Considered as hypersensitivity small vessels vasculitis.
In most of cases, there is a history of preceding URTI.
Age: 2 – 10 years.
M>F.
ITP

34. ITP HSP: C/P: Skin rash: Purpuric rash. Distributed mainly on
the lower limb and
buttock.

35. ITP HSP: C/P: Joints: Arthritis. Abdomin: Abdominal pain.
Bleeding: hematemesis or melena.
Intussusception.
Renal:
Edema, HTN, hemturea, oligurea.
Normal serum complements.
ITP

36. HSP:
C/P:
CNS:
RARE.
Convulsion.
Stroke.
Facial palsy,..
ITP

37. ITP HSP: Lab Findings: Platelets count: normal. BT, PT & aPTT: Normal.
Serum complements: Normal.
↑ serum IgA.
Ocuult blood in stool.
US abdomen: intusseception.
Urine analysis & RFTs.
ITP

38. Renal functions should be followed up to 1 year after recovery.
HSP:
Treatment:
Analgesic:
to control abdominal pain and arthritis.
Prednisolon:
ITP
Renal functions should be followed up to 1 year after recovery.

39. HSP:
Prognosis:
Complete recovery is the rule unless severe renal disease occurs.
ITP

41. Von Willebrand’s Disease

42. vWD vWD: Quantitative or qualitative defect in vWF.
vWF has two major functions:
Needed for platelets adhesion.
Carries factor VIII.
vWD
Therefore,
vWD affects
both
primary &
secondary
hemostasis.
Therefore, vWD affects both primary & secondary hemostasis.

43. vWD vWD: Classification: Type I TypeII Type III Autosomal Dominant.
Mild quantitative defect.
The most common.
Type I
qualitative defect.
vWF dysfunction
TypeII
severe total quantitative defect.
No vWF produced.
Type III
Autosomal Dominant.
Autosomal Dominant.
Autosomal Recessive.

44. vWD vWD: C/P: Mucosal and cutaneous bleeding. Easy bruising.
Epistaxis.
Menorrhagia.
In severe cases:
Soft tissue hematomas,
GI bleeding.
Hemarthroses.
vWD

45. vWD vWD: Investigations: Platelets count: normal. BT: increased.
PTT: prolonged.
VIII: decreased.
vWD

46. vWD:
Treatment:
Desmopressin (DDVP).
Factor VIII concentrate.
vWD

47. Disorders of Secondary Hemostasis

48. Disorders of Secondary Hemostasis
Congenital
Acquired
Hemophilia
Liver diseases.
Vitamin K deficiency.
DIC.
Present with deep bleeding: Joint, muscles, GI tract, GU tract,
Excessive prolonged post-traumatic bleeding.

49. Hemophilia

50. Hemophilia:
X-linked Recessive.
Hemophilia

51. Hemophilia Hemophilia: C/P: Deep bleeding: 5Hs: Hemarthrosis.
Hematoma.
Hematochezia.
Hematuria.
Head hemorrhage.
Easily bruising.
Prolonged wound bleeding…
Hemophilia

52. Hemophilia Hemophilia: Types: Autosomal Recessive X-Linked Recessive
Factor VIII deficiency.
Hemophilia A
Factor IX deficiency.
“Christmas disease”
Hemophilia B
Factor XI deficiency.
Hemophilia C
X-Linked Recessive
Autosomal Recessive

53. Hemophilia Hemophilia: Investigations: aPTT: prolonged.
PT (& INR): Normal.
Factor assay.
Hemophilia

54. Hemophilia Hemophilia: Treatment: Hemophilia A: Desmopressin (DDVP).
Recombinant Factor VIII.
Anti-fibrinolytic agents (e.g. tranexamic add)
Fresh frozen plasma???
Hemophilia

55. Hemophilia Hemophilia: Treatment: Hemophilia B: Recombinant Factor IX.
Anti-fibrinolytic agents (e.g. tranexamic add)
Fresh frozen plasma.
Hemophilia

56. Hemophilia Hemophilia: Treatment: Hemophilia C: Recombinant Factor XI.
Anti-fibrinolytic agents (e.g. tranexamic add)
Fresh frozen plasma.
Hemophilia

58. Approach a Patient with Purpura

59. Purpura Definition of Purpura:
Red, nonblanching maculopapular lesions.
Caused by intradermal capillary bleeding.
Purpura

60. Causes:
Caused by disorders affecting platelets or blood vessels.
Purpura

61. History History: Age ? Onset? Acute vs. Chronic.
Bleeding in other site:
Mucus membranes.
Joints.
Orifices.
Easily bruising.
Prolonged wound bleeding.
Associated symptoms:
Abdominal pain.
Bloody stool.
Hematemesis.
Hematurea.
Joints pain.
History

62. History History: Associated symptoms: Fever. Bone pain.
Polyurea? Oligurea?
Lethargy?
Headache?
Symptoms of anemia?
Appetite?
Weight loss?
Hx. of recent URTI “during last 3 – 4 weeks”.
Drug use
Family history
Maternal history
Social history
History

63. Physical Examinations
Genaeral Look.
Vital signs.
Growth parameter.
Physical Examinations

64. Physical Examinations
Characteristic of rach:
Distribution.
Color.
Size.
Palpable?
Blachable/Non Blanchable.
Bleeding in mucus membrane e.g., gum,..
Nose? Epistaxis.
Pallor?
Physical Examinations

65. Physical Examinations
Retinal hemorrhage?
Joint examination.
Lymph nodes?
Abdominal examination:
Tenderness?
Organomegally?
Rectal examination.
Physical Examinations

66. Investigations Lab.: CBC. Coagulation profile. PT, INR. aPTT. TT.
Factor assay?
Biopsy of rash:
IgA deposits “ Leukocytoclastic vasculitis” --- HSP.
BM:
If suspect leukemia; orgaanomegally,bone pain, lymphadenopathy, prolonged fever,…
Investigations