Bleeding disorders and their management

2. BLEEDING DISORDERS
AND THEIR
MANAGEMENT
Dr. Eaketha Lokesh
PG 1ST Year
Dept: of Prosthodontics
KMCT Dental College

3. CONTENTS
• Introduction
• Bleeding disorders
• History
• Hemostasis
• Screening test
• INR
• Vessel defects

4. CONTENTS
• Introduction
• Bleeding disorders
• History
• Hemostasis
• Screening test
• INR
• Vessel defects

5. • Platelet disorders
• Coagulation disorders
• WBDR
• Recent advancements
• Summary
• References

6. • Vascular damage results in initiation of clotting
with the goal of producing a localized platelet or
fibrin plug to prevent blood loss
• This action is followed by processes that lead to
clot containment, would healing, clot dissolution,
and tissue regeneration and remodeling.
INTRODUCTION

7. BLEEDING DISORDERS???
• caused by the abnormalities of hemostasis and /
or coagulation
• characterized by local or extensive skin or
mucocutaneous hemorrhage derived from
capillary bleeding is usually spontaneous or from
slight trauma

8. • 1800 - U.S., the transmission of hemophilia from
mothers to sons.
• 1803, a Philadelphia physician named Dr. John
Conrad Otto wrote an account of "a hemorrhagic
disposition existing in certain families."
HISTORY

9. • The word "Hemophilia" first appeared - University
of Zurich in 1828
• 1960s - clotting factors were identified and named.

10. • 1950s , early 1960s - hemophilia and other
bleeding problems were treated with whole blood
or fresh plasma.
There wasn't enough of the factor VIII or IX
proteins in these blood products to stop serious
internal bleeding.

11. • 1960 - cryoprecipitate by Dr. Judith
Pool
• 1960s , early 1970s - concentrates
containing factor VIII and IX (freeze-
dried powdered concentrates)
• 1990s - modern treatment-safer factor
concentrates

12. • Vessel constricts
• Circulating platelets adhere to the vessel
• An intricate series of enzymatic reactions
involving coagulation proteins
• produces fibrin to form a stable haemostatic plug
BLOOD VESSEL INJURY TRIGGERS THE
FOLLOWING SEQUENCE:

14. HEMOSTASIS
Haemostasis or normal blood clotting is
essential for survival
process which causes bleeding to stop

15. Vascular phase
Platelet phase
Coagulation phase
Fibrinolytic phase

16. • Primary hemostasis -platelet plug formation at
sites of injury
• Secondary hemostasis - plasma coagulation
system reaction resulting in fibrin formation
• Primary and secondary hemostasis are closely
linked

17. • When a blood vessel is damaged, results in
vasoconstriction
VASCULAR PHASE

18. • Platelets adhere to the damaged surface and form
a temporary plug
PLATELET PHASE

19. • Through two separate pathways the conversion of
fibrinogen to fibrin is complete
COAGULATION PHASE

21. • Anticlotting mechanisms are activated to allow clot
disintegration and repair of the damaged vessel
FIBRINOLYTIC PHASE

22. HEMOSTASIS DEPENDENT UPON :
Vessel Wall Integrity
Adequate Numbers of Platelets
Proper Functioning Platelets
Adequate Levels of Clotting Factors
Proper Function of Fibrinolytic Pathway

23. 1. Platelet count
2. Bleeding time
3. Prothrombin time
4. Partial thromboplastin time
5. Thrombin time
SCREENING TEST

24. • Hemostasis, capillary and platelet function
• Normal 150,000 - 450,000 cells/mm 3
• < 150,000 thrombocytopenia
50,000 - 100,000 mild thrombocytopenia
50,000- severe thrombocytopenia
• ˃ 450,000 cells/mm3 thrombocytosis
1. PLATELET COUNT

25. • Provides assessment of platelet count and
function
• Normal value 2-8 minutes
II. BLEEDING TIME

26. • Measures effectiveness of the extrinsic pathway
• Normal value 10-15 secs
used to determine the clotting tendency of blood
in the measure of warfarin dosage
liver damage
vitamin K status
III. PROTHROMBIN TIME

27. • PT measures factors II , V, VII, X and fibrinogen .
• used in conjunction with the activated partial
thromboplastin time (aPTT) which measures the
intrinsic pathway .

28. • Measures effectiveness of the intrinsic pathway
• Normal value 25-40 secs
IV. PARTIAL THROMBOPLASTIN TIME

29. • Time for thrombin to convert fibrinogen to fibrin
• A measure of fibrinolytic pathway
• Normal value 9-13 secs
V. THROMBIN TIME

30. • Preferred test of choice for patients taking vitamin
K antagonists (VKA).
• To assess the risk of bleeding or the coagulation
status of the patients.
Patients taking oral anticoagulants are required to
monitor INR to adjust the VKA doses
because these vary between patients.
INTERNATIONAL NORMALIZED RATIO (INR)

31. • The INR is derived from prothrombin time (PT)
• Standardized for the potency of the thromboplastin
reagent
• Developed by the World Health Organization
(WHO) using the following formula:
INR = Patient PT ÷ Control PT
• Dimensionless
• Ranges from a score of 0.9-1.3
Tripathi et al . Clinical evaluation of whole blood prothrombin time (PT) and
international normalized ratio (INR) using a Laser Speckle Rheology sensor. Sci

32. • For normal patients who are not on anticoagulation, the
INR is usually 1.0 regardless of the ISI or the particular
performing laboratory
• High INR level (INR=5) high chance of
bleeding
• INR=0.5 high chance of having a clot
Tripathi et al . Clinical evaluation of whole blood prothrombin time (PT) and international
normalized ratio (INR) using a Laser Speckle Rheology sensor. Sci Rep. 2017 Aug

33. WHAT CAUSES
BLEEDING
DISORDERS???

34. 1. Vessel defects
2. Platelet disorders
3. Factor deficiencies
4. Other disorders
CAUSES OF BLEEDING DISORDERS
(National Hemophilia Association)

35. Vessel defects
1. Vitamin C
deficiency
2. Bacterial & viral
infections
3. Acquired
Platelet disorders
1. Thrombocytopeni
a
2. Thrombocytopath
y
Factor
deficiencies
(congenital)
1. Hemophilia A
2. Hemophilia B
3. Von
willebrand’s
disease
Other disorders
(acquired)
1. Oral anticoagulants
(coumarin, heparin)
2. Liver disease
3. Malabsorption
4. Broad spectrum
antibiotics

36. VASCULAR DISORDERS
Petechiae,
purpura,
ecchymosis
Senile purpura
Vitamin C
deficiency (Scurvy)
Connective tissue
disorders
Henoch-Schonlein
purpura

37. Tiny blood vessels
(capillaries) link the
smallest parts of your
arteries to the smallest
parts of your veins.

38. • Small (1–2 mm) red or purple
spot on the skin, caused by a
minor bleed from
broken capillary blood vessels
• Petechiae appear when
capillaries bleed, leaking blood
into the skin
1. PETECHIAE

40. • Cytomegalo virus
infection
• Endocarditis
• Meningococcemia
• Mononucleosis
• Rocky spotted fever
• Scarlet fever
• Sepsis
• Strep throat
• Viral haemorrhagic
fevers
• Vasculitis
• Thrombocytopenia
• Leukemia
• Scurvy (vitamin C
deficiency)
• Vitamin K deficiency
INFECTIUOS DISEASES NON-INFECTIUOS DISEASES

41. • the appearance of red or purple discolorations on
the skin that do not blanch on applying pressure
• They are caused by bleeding underneath the skin
2. PURPURA

42. The medical term for a
subcutaneous hematoma
larger than 1 centimeter,
commonly called a BRUISE
both in the skin as well as in
a mucous membrane
3. ECCHYMOSIS

43. In the purpuric disorders, petechiae commonly are
associated with multiple superficial ecchymoses,
which usually develop without perceptible trauma
but seldom spread into deeper tissues.
Small isolated ecchymoses are commonly noted
in apparently normal women, especially on the
legs, and in small children.

44. 1-2 mm
≥ 3 mm
˃ 1-2 cm

45. • Bleeding into joint spaces.
• Most seen in hemophilia A or hemophilia B
• Rare in disorders of the vessels and platelets or in
acquired coagulation disorders.
4. HEMARTHROSIS

46. • common, benign condition
• characterised by the recurrent formation of
purple ecchymoses (bruises) on the extensor
surfaces of forearms following minor trauma
5. SENILE PURPURA

47. • It is also known as Bateman purpura, after
British dermatology pioneer Thomas Bateman,
who first described it in 1818;
• and actinic purpura, because of its association
with sun damage.
(Senile Purpura. Ken et al. DermNet NZ 2014)

48. RISK FACTORS
oral or topical corticosteroids

49.  Characterised by irregularly-shaped macules, 1 –
4 cm in diameter, that are dark purple with well-
defined margins.
The lesions do not undergo the colour changes of
a bruise and take up to three weeks to resolve.

50. Surrounding skin is typically thin, inelastic and
pigmented in association with others signs of skin
ageing and sun damage.
Extensor surface of forearms and dorsal aspect of
hands
Infrequently, they also occur on necks and faces

51. • benign and self-resolving
• Patients should be educated on sun
protection measures, includes
sunscreen application and sun-protective clothing
to protect their skin from further photodamage
MANAGEMENT

52. • Vitamin C (ascorbic acid)
• Humans can not synthesize vitamin C due to
deficiency of l-gulonolactone oxidase (rich in citrus
food)
• Deficiency- results in SCURVY
6. VITAMIN C DEFICIENCY

53. • Elderly patients seem to require vitamin C in large
quantities.
• They feel better and are able to do more work
when vitamin C is added to their diet.
• Vitamin C is nontoxic even in massive doses.

54. National Academy of Sciences (RDA)
Recommended Dietary Allowances in 2000 for vitamin
C
75+age male → 50mg
 Female → 30mg
Young individuals (male = female)→ 40-30mg

55. proline
5-hydroxyproline
lysine
5-
hydroxylysine
Vitamin C
Hydroxylases
Mirhadi SA et al., 1990
AMINOACIDS IN COLLAGEN

56. The protective role of vitamin C can help our
skin and gums from pinpoint hemorrhage
(scurvy disease).
Cardiovascular diseases, cancers, and joint
diseases are all associated with vitamin c
deficiency and can be partly prevented by
optimal intake of vitamin c.
Kurl S et al, 2002

57. spongy and swollen gumsloose teeth
Cork screw hair pattern with tiny
bleeding points around orifice of a hair
follicle
Woody legs with large
spontaneous bruises in
lower extremities.
delayed wound healing

58. Vitamin C achieves much of its
protective effect by functioning as an
antioxidant and preventing oxygen-
based damage to our cells.
Structures that contain fat (like the
lipoprotein molecules that carry fat
around our body) are particularly
dependent on vitamin C for protection
Reaven PD, Witztum JL:
1996

59. • disorder that causes inflammation
and bleeding in the small blood
vessels of skin, joints, intestines and
kidneys.
• The most striking feature - purplish
rash, typically on the lower legs and
buttocks
• Children: ages of 2 and 6
7. HENOCH-SCHONLEIN PURPURA

60. Rarely serious kidney
damage can occur

61. PLATELET
DISORDERS

62. • Bleeding sites localized to superficial sites such as
the skin and mucous membranes
• Immediately after trauma or surgery
• Readily controlled by local measures
BLEEDING FROM A PLATELET
DISORDER

63. QUANTITATIVE
Abnormal distribution
Dilution effect
Decreased production
Increased destruction
QUALITATIVE
Inherited disorders
(rare)
Acquired disorders
Medications
Chronic renal failure
Cardiopulmonary
bypass
PLATELET DISORDERS

64. • Defined as reduced in the platelet count ˂150,
000µL
spontaneous bleeding
prolonged bleeding time
normal PT and PTT
1. THROMBOCYTOPENIA

65. THE RISK OF BLEEDING DEPENDS ON THE
LEVEL OF THE PLATELET COUNT:
Mild
thrombocytopeni
a (platelet ˂150
000 cells/µL)
Moderate
thrombocytopeni
a (platelet 20 000
- 50 000 cells/µL)
Severe
thrombocytopeni
a (platelet ˂ 20
000 cells/µL)

66. ETIOLOGY
Decreased
production of
platelets
Increased
platelets
destruction
Dilutional
Sequestration
Congenital
(Fanconianae
mia, Wiskot-
Aldrich
Syndrome
Acquired
(aplastic
anaemia,
marrow
infiltration, drug
induced )
Immunologic
destruction (ITP,
SLE , Alloimmune
neonatal
thrombocytopenia)
Non-
immunologic
destruction
(HUS, TTP,
DIC, CHD)
Hypersplenis
m
Massive
blood
transfusion

67. • Bruising
• Petechiae
• Purpura
• Mucosal bleeding (epistaxis and gum bleeding)
• Major haemorrhage (severe GI bleeding,
intracranial bleeding or haematuria is less
common)
• Normal platelet count may present in platelet
dysfunction
SIGN AND SYMPTOM

68. • IMMUNE THROMBOCYTOPENIA
• may occur when the immune system mistakenly
attacks platelets
• In children, it may follow a viral infection
• In adults - chronic
• Low platelet count
• Easy bruising
IDIOPATHIC THROMBOCYTOPENIC PURPURA
(ITP)

69. • There are two clinical subtypes of ITP:
• Acute ITP
• Chronic ITP (starts after the disease has been
present for & ˃6 months)

70. Increased destruction of platelets – Thrombocytopenia
Phagocytosis of antibody-coated platelets by the
reticuloendothelial system.
Autoantibodies (IgG or IgM) directed against platelet
membrane antigens (especially glycoprotein complex
IIb/IIIa).
Inappropriate immune recovery follows an acute viral
infection in children.
PATHOGENESIS OF ITP

71. • acute ITP - sudden onset
• chronic ITP - insidious onset
• Petechiae or purpura feet, legs, arms, and
buttocks.
• Mucosal bleeding.
• Palatal petechiae, epistaxis, hematuria,
menorrhagia, GI bleeding.
• Rarely, intracranial hemorrhage may occur in long
standing severe thrombocytopenia.
CLINICAL MANIFESTATIONS

72. • Not all children with acute ITP need
hospitalization.
• Treatment is indicated if there is:
Life threatening bleeding episode (e.g. intra
cranial haemorrhage) regardless of platelet count.
Platelet count ˂20,000/mm³ with mucosal
bleeding.
 Platelet count ˂10,000/mm³with any bleeding.
MANAGEMENT OF ITP

73. Choice of treatment:
• Oral prednisolone - 4 mg/kg/day for 7 days,
taper and discontinue at 21 days.
• IV Methylprednisolone - 30 mg/kg/day for 3 days.
• IV Immunoglobulin - 0.8 g/kg/dose for 1 day OR
250 mg/kg for 2 days.
• IV Anti-Rh(D) immunoglobulin - (50 – 75 µ/kg) in
Rhesus positive patients – may cause haemolytic
anaemia

74. • Splenectomy is only for life threatening in acute
ITP
• For chronic ITP:
 Repeated treatment with IV immunoglobulin or IV
anti-D or high dose pulse steroids are effective in
delaying the need for splenectomy
Splenectomy is effective in inducing remission in
70-80% of childhood chronic ITP

75. • Intracranial hemorrhage - 50% mortality rate
• Risk of ICH highest in:
Platelet count ˂ 20 000/mm³
History of head trauma
Uses of aspirin (inhibitor of platelet aggregation)
Presence of cerebral arteriovenous malformation
• 50% of all ICH occurs after 1 month of
presentation, 30% after 6 months
COMPLICATION

76. • clinical syndrome characterized by progressive
renal failure that is associated with
microangiopathic hemolytic
anemia and thrombocytopenia.
• HUS is the most common cause of acute kidney
injury in children
HEMOLYTIC UREMIC SYNDROME
(HUS)

77. • Common in infants, young children and pregnant
women
• Related to TTP in which
number of platelets suddenly decreases
RBC are destroyed
kidney stop functioning
Schiocytes
thrombocytopenia

78. • HUS is rare, but can occur with certain bacterial
infection (E.coli or shigelladysenteriae)
with the use some drugs (quinine, cyclosporine,
mitomycin C)
Toxin producing organism such as E.coli cause
endothelial damage that activates localized
clotting, leading to platelet aggregation and
consumption

79. Mainly supportive
Dialysis
No antibiotics
Plasmapheresis / i.v IgG
TREATMENT

80. • Resembles hemolytic uremic syndrome but occur
more commonly in adults than in children
• Spontaneous aggregation of platelets and
activation of coagulation in small blood vessels
• In TTP, platelet consumption, precipitated by a
congenital or acquired deficiency of
metalloproteinase that cleaves vWF
THROMBOTIC THROMBOCYTOPENIC
PURPURA (TTP)

81. FEATURES TTP HUS
Onset Young adult Infants and children
Predisposing factors Following stem cell
transplantation
(donor is unrelated)
Following
gastroenteritis- E.coli
Clinical findings  Microangiopathic
hemolytic
anemia
 Thrombocytopeni
a
 Renal
dysfunction -
hematuria
C/F  Neurologic
disturbances
 Fever
• Abdominal pain
• Bloody diarrhea

82. • Progressive, malignant disease of the blood
forming organs
• Marked by distorted proliferation and
development of leukocytes and their precursors
in the blood and the bone marrow
MARROW INFILTRATION
(LEUKEMIA)

83. • Overproduction of these white cells(immature or
abnormal forms) → suppresses the production of
normal WBC, RBC and platelets
• Lead to increase susceptibility to infection ,
anemia and bleeding

84. • Result from infiltration of bone marrow or other organs with
leukemic blast cells
CLINICAL PRESENTATION

86. • Progress rapidly in some children
• Blood count is abnormal,
• low hemoglobin
• thrombocytopenia
• evidence of circulating blast cells in most children
Bone marrow examination to confirm the
diagnosis

87. • a condition in which blood clots form throughout
the body, blocking small blood vessels
• Serious medical condition
• Occurs as secondary complication of variety
diseases.
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)

88. Caused by the systemic
activation of coagulation
pathways → formation of
thrombi throughout the
microcirculation and
widespread thromboses.

89. • May be acute or chronic.
• Initiated through the TISSUE FACTOR PATHWAY
• Consumption of platelets and coagulation factors
• Secondarily activation of fibrinolysis
• As consequence, there is depletion of the
elements required for hemostasis ( consumptive
coagulopathy)

90. • Blood transfusion reaction
• Cancer, especially certain
types of leukemia
• pancreatitis
• Infection in the blood (bacteria
or fungus)
• Liver disease
CAUSES OF DIC*
* Medical encyclopedia

91. • Pregnancy complications (such as
placenta that is left behind after
delivery)
• Recent surgery or anesthesia
• Severe tissue injury (as in burns and
head injury)
• Large hemangioma (a blood vessel that
is not formed properly)
* Medical encyclopedia

92. The commonest causes of
activation of coagulation are
severe sepsis or shock due to
circulatory collapse, e.g in
meningococcal septicemia or
extensive tissue damage from
trauma or burn

93. INFECTIOUS
 Meningococcemia
 Other gram –vebact (Salmonella, E.coli,
Haemophilus)
 Virus (CMV, herpes, hemorrhagic
fevers)
 Rickettsia, Malaria and fungus
TISSUE INJURY
 CNS trauma
 crush injury
 Multiple fracture with fat emboli
 Profound shock of asphyxia
Hypothermia/hyperthermia
 Massive burns
MALIGNANCY
 Acute promyelocytic leukemia
 Acute monoblastic or myelocytic
leukemia widespread malignancies
(neuroblastoma)
HEREDITARY THROMBOTIC
DISORDERS
 Antithrombin III def.
 Homozygous protein C def.
MICROANGIOPATHIC DISORDER
 Severe TTP/ hemolytic uremic
syndrome
 Giant hemangioma
VENOM/ TOXIN
 Snake bites
 Insect bites
GIT DISORDER
 Fulminant hepatitis
 severe IBD
 Reye syndrome
NEWBORN
 Maternal toxemia
 G. B strep. Infection
 Abruptioplacentae
 Congenital viral disease (CMV,
herpes)
MISCELLANEOUS
 Severe acute graft rejection
 Acute hemolytic transfusion reaction
 Heparin induced thrombosis

95. • Treatment of underlying cause
• Possibly replacement therapy
TREATMENT APPROACHES
anticoagulation

96. • Immediate correction of the cause is the priority
(eg, broad-spectrum antibiotic treatment of
suspected gram-negative sepsis, evacuation of
the uterus in abruptio placentae).
• If treatment is effective, disseminated
intravascular coagulation should subside quickly.

97. • any of several blood disorders characterized by
dysfunctional platelets (thrombocytes), which
result in prolonged bleeding time, defective clot
formation, and a tendency to hemorrhage.
• Denotes abnormal platelet function
• Platelet count normal
• Thrombocytopathy may be congenital or acquired
2. THROMBOCYTOPATHY

98. INHERITED
• Von willebrand disease
• Thrombasthenia
• Bernard-soulier
syndrome
• Down syndrome
• Wiskott-aldrich
syndrome (an immune
disorder).
ACQUIRED
• Leukemia
• Pernicious anemia
• Scurvy
• uremia
THROMBOCYTOPATHY
https://www.britannica.com/editor/The-Editors-of-Encyclopaedia-
Britannica/4419

99. • Platelet transfusion to control bleeding
• Curing of the underlying disease usually results in
improved platelet function
MANAGEMENT

101. BLEEDING DISORDERS
AND THEIR
MANAGEMENT
Eaketha Lokesh
PG 1ST Year

102. CONTENTS- I
• Introduction
• Bleeding disorders
• History
• Hemostasis
• Screening test
• INR
• Vessel defects
• Platelet disorders

103. • Coagulation disorders
• Prosthetic considerationssssssss
• WBDR
• Recent advancements
• Summary
• References
CONTENTS- II

104. COAGULATION
DISORDERS

105. • Bleeding sites: in deep subcutaneous tissues,
muscles, joints, or body cavities
• time: hours or days after injury
• Unaffected by local therapy
BLEEDING FROM COAGULATION DEFECTS

106. Hemophilia A
Hemophilia B
Von Willebrand disease
parahemophilia

107. • HEMOPHILIA ----------"Royal disease"
• Hemophilia gene was passed from Queen
Victoria, who became queen of England in 1837,
to the ruling families of Russia, Spain, and
Germany.
HEMOPHILIA

109.  Factor VIII (FVIII) deficiency
CLASSIC HEMOPHILIA
A genetic disorder caused by missing or defective
factor VIII, a clotting protein.
Although it is passed down from parents to
children, about 1/3 of cases are caused by a
spontaneous mutation, a change in a gene
HEMOPHILIA A

111. Incidence:
approximately 1 in 5,000 live births
(US Centers for Disease Control and Prevention)
X-linked recessive

112. • Normal plasma levels of FVIII range from 50% to
150%
• Levels below 50%, or half of what is needed to
form a clot, determine a person’s symptoms.
Mild hemophilia A- 6% up to 49% of FVIII in the
blood
Moderate hemophilia A- 1% up to 5% of FVIII in
the blood
Severe hemophilia A - <1% of FVIII in the blood

114. How frequently a person bleeds
and the severity of those bleeds
depends on how much FVIII is in
the plasma, the straw-colored
fluid portion of blood

115. MANAGEMENT

116. Avoid certain drugs that can
aggravate bleeding problems:

117. I. Factor VIII products for patients who are HIV
seronegative, including Recombinant factor VIII
II. Immunoaffinity purified factor VIII concentrates
III. Cryoprecipitate is not recommended because of
the risk of HIV and hepatitis infection.
IV. Mild hemophilia A should be treated with
desmopressin, in a DDAVP injection or Stimate
nasal spray
MASAC made recommendations for treatment of
hemophilia in November of 1999

118. COMPLICATIONS
 Chronic joint deformities
 Intracerebral
hemorrhage

119. • CHRISTMAS DISEASE
• deficiency in clotting factor IX
• Hemophilia B is four times less common than
hemophilia A
• X-linked recessive
• Incidence: 1 in 35000 men
HEMOPHILIA B

120. • Severe (factor levels less than 1%) represent
approximately 60% of cases
• Moderate (factor levels of 1-5%) represent
approximately 15% of cases
• Mild (factor levels of 6%-30%) represent
approximately 25% of cases

122. • Nose bleeds
• Bruising
• Spontaneous bleeding
• Bleeding into joints and associated pain and
swelling
• Gastrointestinal tract and Urinary tract
hemorrhage
• Blood in the urine or stool
• Prolonged bleeding from cuts, tooth extraction,
surgery, following circumcision
SYMPTOMS

123. • Infusing the missing clotting factor
• To prevent a bleeding crisis- taught to administer
factor IX concentrates at home at the first signs of
bleeding
• Factor IX concentrate may be given prior to dental
extractions and surgery to prevent bleeding
TREATMENT

124. Milder forms of
hemophilia need to
have dental or other
surgery, the drug
desmopressin acetate
(DDAVP) may be
given to improve
clotting temporarily so
that transfusions can
be avoided.

125. Recombinant factor IX products for patients who
are HIV seronegative
Patients who are HIV-seropositive should also be
treated with high purity products such as
immunoaffinity purified and recombinant factor VIII
products.
MASAC MADE RECOMMENDATIONS FOR
TREATMENT OF HEMOPHILIA B IN NOVEMBER OF
1999

126. For patients with inhibitors to
factors VIII and IX, there is
Recombinant Factor VIIa
(NovoSeven)
Produced by baby hamster
kidney cells, no human
albumin or other proteins are
used in its production, reducing
virus risk.

127. There is also Porcine factor VIII (Hyate C) and
activated prothrombin complex concentrates
Not currently available

128. hereditary
deficiency or
abnormality of the
von Willebrand
factor in the blood,
a protein that
affects platelet
function
VON WILLEBRAND DISEASE

129. • Type I:
Most common and mildest form of von Willebrand
disease.
Levels of von Willebrand factor are lower than
normal, reduced levels of factor VIII.
CLASSIFICATIONS

130. • Type II:
• Von Willebrand factor itself has an abnormality.
• Depending on the abnormality, they may be
classified as:
Type II a - the level of von Willebrand factor is
reduced, as is the ability of platelets to clump
together.
Type II b -although the factor itself is defective,
the ability of platelets to clump together is actually
increased.

131. • Type III:
Severe von Willebrand disease.
Total absence of von Willebrand factor, and
 factor VIII levels are often less than 10%

132. • Pseudo (or platelet-type) von Willebrand disease:
Resembles Type IIb von Willebrand disease,
but the defects appears to be in the platelets,
rather than the von Willebrand factor.

133. Inheritance Pattern occurs in men and women
equally.
Types I and II - inherited as a "dominant" pattern.
Type III von Willebrand disease - inherited in a
"recessive" pattern

134. Normal platelet count
Prolonged bleeding time
Reduced von willebrand factor level
Reduced platelet adhesion may occur
Reduced or increased platelet aggregation
(platelet aggregation test)
Ristocetin cofactor is reduced.
DIAGNOSIS

135. Stimate, desmopressin acetate (DDAVP),nasal
spray or injection
Viral-inactivated factor VIII preparations rich in
von Willebrand factor, such as Alphanate,
Humate-P and Koate DVI, are recommended
Cryoprecipitate is not recommended except in
life-threatening emergencies because of the risk of
HIV and hepatitis infection
MASAC MADE RECOMMENDATIONS FOR
TREATMENT OF VON WILLEBRAND DISEASE IN
NOVEMBER OF 1999

136. After surgery, hemorrhaging may occur.
The condition is worsened by the use of aspirin
and other nonsteroidal anti-inflammatory drugs.
 Women may have risks during pregnancy and
childbirth.

137. PROSTHETIC
CONSIDERATIONS
IN BLEEDING
DISORDERS

138. • Good thorough medical
history- family history ,
personal history, medications,
spontaneous bleeding
• Physical examination
• Screening clinical lab tests
• Excessive bleeding following
surgical proceedure
DENTAL EVALUATION

139. Aspirin
Anticoagulant
Alcohol
Antibiotics
anticancer
REVIEW OF MEDICATIONS
5
A

140. 1. Removable prosthodontics dentures
donot usually involve a considerable risk
of bleeding.
2. Trauma should be minimized by careful
post-insertion adjustments.
3. Oral tissues should be handled delicately
during the various clinical stages of
prosthesis fabrication to reduce the risk
of eccymosis

141. • Only global registry collecting standardized clinical
data on people with hemophilia from around the
world
WBDR

142. • New clotting products and drugs - desmopressin
acetate also known as DDAVP.
• Synthetic (not derived from plasma) clotting
products that take advantage of recombinant
technologies.
• Better screening methods to detect and remove
viruses and other agents from factor concentrates
and blood products
RECENT ADVANCEMENTS

143. • Improved surgical options
• Advanced genetic testing methods
• Medically supervised home-infusion therapy
• Prophylactic treatment

144. SUMMARY

145. • National hemophilia foundation
• The worldwide incidence of hemophilia is
estimated at more than 400,000 people. 108 Kar,
A., Phadnis, S., Dharmarajan, S., & Nakade, J.
(2014). Epidemiology & social costs of
haemophilia in India. The Indian Journal of
Medical Research, 140(1), 19–31.
REFERENCES

146. • Davidson’s “principles and practice of medicine-
19th edition;- Hanslet, Chilvers, Boon, Colledge,
Hunters.
• Medical emergencies in the dental practice –
malammed; 5th edition
• Bailey and Love’s Short practice of surgery-23rd
edition; Russel, Williams, Bulstrode;

147. • Complications in Oral and Maxillofacial surgery-
1st edition; Kaban, Pogrel, Perrot.
• Concise Medical Physiology;-5th edition;
Chaudhari
• Systemic disease in dental treatment-1st edition;-
Michael.J.Tullman, Spencer.W.Redding.
• Clinical hematology-7th edition;-
Maxwell.M.Wintrobe.