Hemophilia in dental clinic and how to manage

1. Child has diagnosis of
HEMOPHILIA in
dental clinic
Dr Hussein H. Abdeldayem, MD
Professor of Pediatrics
Pediatric Neurology Consultant

2. CASE I
 18 month old child presents with bleeding
from frenulum. Injury to mouth four days
ago. Bleeding has stopped on several
occasions but keeps recurring. Family
doctor send him to you for suggestions
Patient had no other surgery

3. Workup
 􀂍 History
 􀂍 History
 􀂍 History
 􀂍 Physical exam
 􀂍 Laboratory tests

4. Bleeding Disorders & Dentistry
 Does the patient have bleeding with
a dental procedure that is not normal and
deserves referral for evaluation?

5. CASE II
 14 year old young children who had
extraction of a molar teeth
 Bleed for several days after the procedure
with impressive bruising or face and neck
 History of bleeding with loss of primary teeth,
bruising, epistaxis

6. Patient has diagnosis
of HEMOPHILIA

7. (typical of
coagulation factor
disorders)

9. Hemophilias
Hemophilia A Hemophilia B Hemophilia C
↓FactorVIII ↓Factor IX ↓Factor XI
x linked x linked Autosomal
Recessive
All of them similar in c/p but different in treatment

10. Hemophilia A and B
Hemophilia A Hemophilia B
Coagulation factor deficiency Factor VIII Factor IX
Inheritance X-linked X-linked
recessive recessive
Incidence 1/10,000 males 1/50,000 males
Severity Related to factor level
<1% - Severe - spontaneous bleeding
1-5% - Moderate - bleeding with mild injury
5-25% - Mild - bleeding with surgery or trauma
Complications Soft tissue bleeding

12. Child's Chances of
Hemophilia
FatherMother
50% sons , diseased
50% daughter, carrier
NormalCarrier
All sons normal
All daughter carrier
hemophiliaNormal
50% sons , diseased
Daughter may Hemophilic
HemophiliaCarrier

14. Site of bleeding
3 H 3 serious 3 m.m
- Hemarthrosis - intracranial hge - dental
- Hematoma - intraocular hge - epistaxis
- Hematuria - retroperitoneal hge - GIT

15. Hemophilia
Clinical manifestations (hemophilia A & B
indistinguishable)
Hemarthrosis (most common)
Fixed joints
Soft tissue hematomas (e.g., muscle)
Muscle atrophy
Shortened tendons
Other sites of bleeding
Urinary tract
CNS, neck (may be life-threatening)
Prolonged bleeding after surgery or dental
extractions

16. Treatment of hemophilia A
 Intermediate purity plasma products
 Virucidally treated
 May contain von Willebrand factor
 High purity (monoclonal) plasma products
 Virucidally treated
 No functional von Willebrand factor
 Recombinant factor VIII
 Virus free/No apparent risk
 No functional von Willebrand factor

17. Dosing guidelines for hemophilia A
 Mild bleeding
 Target: 30% dosing q8-12h; 1-2 days (15U/kg)
 Hemarthrosis, oropharyngeal or dental, epistaxis, hematuria
 Major bleeding
 Target: 80-100% q8-12h; 7-14 days (50U/kg)
 CNS trauma, hemorrhage, lumbar puncture
 Surgery
 Retroperitoneal hemorrhage
 GI bleeding
 Adjunctive therapy
 amino caproic acid (Amicar) or DDAVP (for mild disease only)

18. Complications of therapy
 Formation of inhibitors (antibodies)
 10-15% of severe hemophilia A patients
 1-2% of severe hemophilia B patients
 Viral infections
 Hepatitis B Human
parvovirus
 Hepatitis C Hepatitis A
 HIV Other

19. Hge according to factor VIII
< 1 % activity = Severe
1-5 % activity = moderate
5-25 % activity = mild

20. INVESTIGATION
Prolonged clotting time
Prolonged PTT & normal PT
Reduced level of factor VIII

21. Treatment
Avoid
bleeding arrest
specific treatment

22. Avoid
Aspirin
IM injection
Surgery even minor

23. Bleeding arrest
Cold compression
Pressure & immobilization
Application of powdered thrombin to the wound

24. Specific treatment
- Fresh frozen plasma - Cryo precipitate
- Factor VIII concentrate
Orthopeadic surgery for crippled
hemophilia

25. Treatment of hemophilia B
 Agent
 High purity factor IX
 Recombinant human factor IX
 Dose
 Initial dose: 100U/kg
 Subsequent: 50 U/kg every 24 hours

26. VWD
(autosomal dominant)
↑Clotting time ẽ purpra ẽ coagulation
↑ Bleeding time defect
Abnormal function Ex:-
V W Factor Hemarthrosis
Hematoma
Hematuria

27. von Willebrand Disease
Clinical features
 von Willebrand factor Carrier of factor VIII
Anchors platelets to subendothelium
Bridge between platelets
 Inheritance Autosomal dominant
 Incidence 1/10,000
 Clinical features Mucocutaneous bleeding

28. Laboratory evaluation of
von Willebrand disease
Classification
 Type 1 Partial quantitative deficiency
 Type 2 Qualitative deficiency
 Type 3 Total quantitative deficiency
 Diagnostic tests:
vonWillebrand type
Assay 1 2 3
vWF antigen  Normal 
vWF activity   
Multimer analysis Normal Normal Absent

29. Treatment of von Willebrand disease
Varies by Classification Cryoprecipitate
 Source of fibrinogen, factor VIII and VWF
 Only plasma fraction that consistently contains VWF multimers
 Correction of bleeding time is variable
 DDAVP (Deamino-8-arginine vasopressin)
 Increases plasma VWF levels by stimulating secretion from
endothelium
 Duration of response is variable
 Used for type 1 disease
 Dosage 0.3 µg/kg q 12 hr IV
 Factor VIII concentrate (Humate-P)
 Virally inactivated product
 Used for type 2 and 3

30. Clinical History
 􀂍 Bleeding disorders that have already
 been diagnosed
 􀂍 Unrecognized bleeding disorders
 􀂍 Other medical problems or medications
that might affect bleeding

31. Clinical History
 􀂍 Ask specific questions
 􀂍 Sites of bleeding, severity and
frequency, spontaneous or with trauma
 􀂍 Family history
 􀂍 General medical history
 􀂍 Medications

32. Medications
 􀂍 Aspirin
 􀂍 Nonsteroidals
COX-2 inhibitor (Celebrex) - OK
 􀂍 Heparin, coumadin
 􀂍 Psychiatric meds (Zoloft, Prozac, etc.)
 􀂍 Seizure meds (Tegretol, Depakote, etc.)
 􀂍 Other

33. Screening Coagulation Tests
􀂍 Coagulation panel
 PT, PTT, TT
 Platelet count
 Bleeding time vs. platelet function assay
􀂍 Do these help identify bleeding
disorder patients?
a- Severe bleeding disorders
b- Mild/moderate bleeding disorders
􀂍 Further evaluation ?

34. Clinical History
 􀂍 Ask specific questions
 􀂍 Sites of bleeding, severity and
frequency, spontaneous or with trauma
 􀂍 Family history
 􀂍 General medical history
 􀂍 Medications