This document discusses the dental management of patients with hematological disorders. It covers the basics of blood physiology, hemostasis and coagulation factors. Specific disorders discussed in detail include hemophilia A/B which are sex-linked bleeding disorders caused by a deficiency of coagulation factors VIII and IX respectively. The clinical features, investigations, classifications and treatments are outlined for these conditions. Modified dental protocols are recommended to minimize bleeding risks in affected patients, including local hemostatic measures and factor replacement therapies.

1. DENTAL MANAGEMENT OF
PATIENTS WITH
HAEMATOLOGICAL
DISORDERS
Dr.Tinet Mary Augustine. BDS,MDS
Pediatric Dentist
Dr.Tinet’s Pedorayz, Pediatric And Early Age Orthodontic
Dental Clinic DR.TINET MARY AUGUSTINE.BDS.MDS 1

2. INTRODUCTION
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3. BLOOD-BASIC PHYSIOLOGY
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4. HEMOPOESIS
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5. GENETIC BASIS
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6. RED BLOOD CELLS
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7. WHITE BLOOD CELLS
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8. PLATELETS
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9. PLASMA
• 91% WATER
• 7%PLASMA PROTEIN-
ALBUMIN-OSMOTIC
PRESSURE,BUFFERING,TRANSPORT OF FATTY
ACID
GLOBULIN-PROTEIN TRANSPORT,ANTIBODIES
FIBRINOGEN-BLOOD CLOT
• 2% GAS,NUTRIENTS,WASTE PRODUCTS
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10. PREVENTION OF CLOT
• NO,PGI2,ADP-DP
• Heparin Cell Fate And Antithrombin(X,IX)
• Thrombomodulator,thrombin,C Protein(V,VII)
• Tissue Plasminogen Activator(Fibrin)
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11. HEMOSTASIS
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12. CLOTTING FACTORS
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13. DR.TINET MARY AUGUSTINE.BDS.MDS 13

14. HEMOPHILIA
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15. ROYAL DISEASE
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16. HISTORY
• First noticed in the work of Hopff in which he
explained the diases at University of Zurich in
1828
• PAVLOVSKY-noticed the phenomenon.but
couldn’t explain the reason
• 1952 AGGLER and co workers explained the
deficiency of Plasma thromboplastin
component (IX/christmas factor) as the reason
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17. ETIOLOGY
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18. TYPES
HEMOPHILIA A (VIII)
HEMOPHILIA B (IX)
HEMOPHILIA C (XI)
PARAHEMOPHILIA (V)
VON WILLEBRAND DISEASE
X-CHROMOSOME
HEMOPHILIA A-F8
HEMOPHILIA B-F9
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19. HEMOPHILIAA
• X CHROMOSOME LINKED RECESSIVE
• OCCUR IN MALE,FEMALES BEING THE CARRIER
• VII-ACTIVATED BY VIIa AND EFFECTIVE CO
FACTOR FOR Ixa TO ACTIVATE X
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20. HEMOPHILIA B
• AGGLER AND CO WORKERS IN 1952
DISCOVERED
• 300 MUTATIONS WERE REPORTED
• IX IS A VIT K DEPENDENT
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21. CLINICAL FEATURES
EASY BRUISING
PROLONGED BLEEDING
OOZING OF BLOOD AFTER TOOTH EXTRACTION
HEMATOMAS
HEMARTHROSIS(EVEN BY WALKING)
HEMATURIA
MUCOUS MEMBRANE HEMORRHAGE
SEVERE NOSE BLEEDS
BLEEDING IN BRAIN
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22. ORAL MANIFESTATIONS
• HEMATOMAS-BLEEDING INTO TONGUE AND
FRENULUM
• HEMORRHAGIC MARKS IN THE ORAL CAVITY
USUALLY ASSOCIATED WITH ALLERGIC
REACTIONS OR TRAUMA
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23. CLINICAL CLASSIFICATION
• MILD-6-30% (O.O6-0.30 U/ML) spontaneous
hemorrhages are rare
• MODERATE 1-5%(O.O1-O.O5U/ML).occasional
spontaneous hemorrhage can be seen.
• SEVERE less than 1%(O.1U/ML) spontaneous
hemorrhages are seen in early infancy and frequently
therafter. patient need clotting factor replacementDR.TINET MARY AUGUSTINE.BDS.MDS 23

24. INVESTIGATION
• LAB TEST
• PLATELET-NORMAL
• PROTHROMBIN TIME-EXTRINSIC AND
COMMON PATHWAY(FACTOR VII,X,V,II,I)
• PARTIAL THROMBOPLASTIN TIME(TEST
INTRINSIC AND COMMON
PATHWAYS(XII,XI,IX,VIII,X,V,II,I)
NORMAL
PROLONGED
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25. CONFIRMATION
SPECIFIC FACTOR ACTIVITIES
MUTATION TESTING OF GENES
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26. TREATMENT
• GENERAL-AVOID IM INJECTION. USE OF
ASPIRIN AND NSAIDS
• FACTOR VIII REPLACEMENT-INFUSION OF
FRESH FROZEN PLASMAAND
CRYOPRECIPITATE.(MAXIMUM
ACHIEVABLE LEVEL IS 20%)
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27. SEVERE CASES
• MISSING OF MISSING FACTORS
• IN SEVERE-(INTRINSIC FACTORS ARE
ABSENT/LOW) SUPPLEMENTAL FACTORS
CAUSE ANTIBODY PRODUCTIONS WHICH
CAN INHIBIT THE TREATMENT ALL THE
TIME AND CAN CAUSE EVEN ANAPHYLAXIS
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28. DESMOPRESSIN
1 –DEAMINO 8-D ARGININE VASOPRESSIN(DD
AVP)
Helpful For Mild Vii Defeciency
Stimulate Von Willebrand Factor To Release From
Endothelial Cell Which Can Cause Stabilization Of
Residual Factor VIII
In Severe Cases –Do Not Respond
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29. DENTAL CLINICAL
MANAGEMENT
• ANTIFIBRINOLYTIC AGENTS-TO ENHANCE
HEMOSTASIS
• FIBRIN GLUE/FIBRIN TISSUE ADHESIVES
• FACTOR XIII MIXTURE IS PLACED ON
INJURY SITE AND CLOTTED BY THE HELP
OF THROMBIN SOLUTION CONTAINING
CALCIUM
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30. HOME THERAPY USING FACTOR VIII
CONCENTRATS.PATIENTS ARE
PROLONGED WITH ENOUGH SUPPLY.
CHILDREN BETWEEN 3-6 YEARS-MANAGED
WITH HELP OF PARENTS .AND ABOVE 6
WERE TAUGHT TO TAKE BY THEMSELVES
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31. LIVER TRANSPLANT-IN OLDRER
PATIENTS TO CURE HEMOPHILIAAND
ALSO CHRONIC HEPATITIS
GENE THERAPY-GENEREPLACEEMNT
THERAPY USED AS A PROPHYLAXIS AND
AS A TREATMENT
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32. DENTAL MANAGEMENT
• Depends on the severity and the invasiveness
of the procedure
• Medical consultation
• Injections-avoid regional blocks and Im
injections rather go for intra ligamentary or
intraosseous injections.
• Avoid the chances of deep infections
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33. MODIFIED PROTOCOLS FOR
HANDLING HEMOPHILIAC
PATIENTS
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34. DR.TINET MARY AUGUSTINE.BDS.MDS 34

35. IDENTIFYING PATIENTS AT RISK OF BLEEDING
History of:
• Bleeding problems in relatives.
• Bleeding problems after surgery, tooth extraction and
trauma.
• Medications: Aspirin, anticoagulants, long term
antibiotics.
• Illnesses associated with bleeding problems: Leukemia,
congenital heart disease, liver disease, hemophilia
• Spontaneous bleeding
REVIEW
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36. Clinical findings:
• Jaundice, pallor
• Spider angiomas
• Ecchymoses, petechiae
• Oral ulcers
• Hyperplastic gingival tissue
• Hemarthrosis
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37. Screening laboratory tests:
• Quantitative (Platelet Count):
a. Normal Platelet Count-150,000~440,000/mm3
b. Thrombocytopenia-Platelet <150,000/mm3
c. Severe intraop bleeding-Platelet <40,000~70,000/
mm3
d. Spontaneous bleeding-Platelet <20,000/mm3
Minimal recommended preop platelet
>75,000/mm3
In: Duke J, editor. Anesthesia Secrets. 4th ed. Elsevier; 2010.
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38. Qualitative (platelet function):
a. Ivy Bleeding Time-Normally 1~6 mins
b. Closure Time from PFA 100-Normally 60~120
seconds
Little JW, Falace DA, Miller CS, Rhodus NL, editors. Dental Management of the Medically
Compromised Patient. 8th ed.; 2012.
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39. Prothrombin time (PT) and
International normalized
ratio (INR)
Tests intrinsic and common pathways
• Normal value: 25-35 secs
Thrombin time (TT):
Tests the common pathway, thus
the ability to form initial
clot from fibrinogen.
• Normal Value: 9-13 secs
[Control should be run for all the above mentioned
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40. SYSTEMIC DISEASES
CAUSING COAGULATION
DEFECTS
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41. TRANSFUSION PRODUCTS
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42. VON WILLEBRAND DISEASE
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43. THANK YOU
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