2. Dr-Majid Qanavat Ped. Hematologist oncologist Isfahan university -1396
Bleeding Disorders
Dr-Majid Qanavat
Ped. Hematologist oncologist
Isfahan university -1396

3. Approach to bleeding tendency in infants and children

4. VASCULAR PHASE PLATELET PHASE PLASMA PHASE
Coagulation system
VASCULAR PHASE PLATELET PHASE PLASMA PHASE

5. Coagulation system Blood vessel injury Vasoconstriction
Simplistic View of Vascular Injury

9. Practical Approach to a Child With Bleeding HISTORY:
AGE OF ONSET
SEX, Family History
LOCATION I TYPE OF BLEEDING
DURATION OF BLEEDING
MEDICATIONS
ASSOCIAED SYMPOMS
REVIEW OF SYSTEMS

10. Practical Approach to a Child With Bleeding (cont
Practical Approach to a Child With Bleeding (cont.) PHYSICAL EXAMINATION :
PETECHIAE
ECHYMOSES
JOINT BLEED & DEEPSEATED HEMATOMAS HEPATOSPLENOMEGALY
SIGNIFICANT LYMPHADENOPATHY
ACTIVE AND PLAYFUL VS. ILL LOOKING
DYSMORPHIC FEATURE
CAFE-AU-LAIT SPOTS
HEMANGIOMAS

12. Approach to a bleeding patient (cont.)

13. Approach to a bleeding patient (cont.)

14. Approach to a bleeding patient Primary homeostasis
Vascular causes :
"Meningococcemia"

15. Henoch-Schonlein Purpura

16. Hemangiomas Kassalback-Merritt syndrome

18. Coagulation test
The results are as good as the corrected samples collection:
Standards: Time from sample to test:
- PT 24 hours ,
- pTT 4 hours.
Blood/citrate ratio:1/9.

19. Laboratory approach to 2° H. defect

20. Laboratory approach to 2° H. defect

23. Haemostatic disorders which may present with normal coagulation screen and full blood count
Mild van Willebrand disease
Mild haemophilia A or B
Mild factor XI or other single factor deficiency
Factor XIII deficiency
Glanzmann thrombasthenia
Platelet storage pool disease
Platelet release defect
Vitamin C deficiency

24. Platelets Disorders -Acquired-Aspirin; Uremia -Glanzmann's
Functional disorders
-Acquired-Aspirin; Uremia
Inherited
-Glanzmann's
-Bernard-Soulier
-Gray platelet syndrome

25. Thrombocytopenia Absent Radius(TAR)

26. ITP •Most have benign &limited course Treatment options:
1.Conservative - wait & watch
2. Aggressive-
- Steroids
- IVIG
- Rhogam
- Rituximab
- splenectomy

27. Von Willebrand Disease
The most common inherited bleeding disorder Occurs in 1% of the population
Type 1 : mild deficiency of VWF
Type2 : multimer dysfunction
Type3: severe deficiency of VWF

28. Von Willebrand Disease(cont)
DIAGNOSIS:
Prolong PTT & BT
SYMPTOMS:
Epistaxis or gingival bleeding
Menorrhaqra
Post-partum hemorrhage
Post-surgical bleeding
Bleeding post-dental extraction

29. Von Willebrand Disease(cont)

30. 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

36. Extensive bruising of the left forearm and hand in a patient with hemophilia.

37. Hemophilia Arthropathy and Muscle Bleeding

38. Clinical Severity of Bleeding Disorders

39. ... the promise of achieving your potential

40. Thank You For Your Attention
Any Question?