The hemophilia are disorders of hemostasis resulting from a deficiency of a procoagulant. Hemophilia is an inherited bleeding disorder affecting approximately 1 in 7500 males.

1. Bleeding disorders
in dental practice
Dr Alvi Fatima
MDS 1st year
Department of Pediatric Dentistry
1

2. Content
• Hemophilia or Inherited Bleeding Disorder.
• Anemia
• Leukemia
2

3. Introduction
• Hemostasis composed of 4 major events that
occur in a set order following the loss of vascular
integrity:
– Vascular constriction -limits the flow of blood
to the area of injury.
3

4. – Platelet aggregation –Blood platelets clump
when binding to collagen that becomes
exposed following rupture of the endothelial
lining of vessels.
• Blood platelets become activated and aggregate at the
site of injury .
• Upon activation, platelets release ADP and TXA2 (which
activate additional platelets). 4

5. • Clot formation -to insure stability of the initially
loose platelet plug, a fibrin mesh (also called the clot)
forms and entraps the plug.
• Fibrinolysis -the clot must be dissolved in order for
normal blood flow to resume following tissue repair.
The dissolution of the clot occurs through the action
of plasmin
5

6. Screening test for hemostasis
Laboratory test Factor/Function
measured
Associated disorders
Bleeding time Platelets function,
vascular integrity
i. Qualitative
disorders of palates
ii. Von Willebrand’s
disease
iii. Quntitative disorder
of platelets
iv. Aquaried vascular
disorders
Platelets count Quantification of
platelets
i. Thrombocytopenia
ii. Thrombocytosis
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7. Laboratory test Factor/function
measured
Associated disorder
Prothrombin time Evaluation of extrinsic
and common pathway
i. Oral
anticoagulation
therapy
ii. DIC
iii. Liver Disease
Partial
thromboplastin time
Evaluation of intrinsic
and common pathway
i. Parenteral heparin
therapy
ii. DIC
iii. Liver disease
Thrombin time Evaluation of common
pathway
i. Afibrinogenaemia
ii. DIC
iii. Parenteral heparin
therapy
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8. Blood disorder
9

9. Hemophilia
The hemophilia are disorders of hemostasis resulting from a
deficiency of a procoagulant. Hemophilia is an inherited
bleeding disorder affecting approximately 1 in 7500 males.
10

10. Hemophilia A
• Hemophilia A, or classic hemophilia, is a deficiency of factor
VIII, also known as anti -hemophilic factor.
• Factor VIII deficiency is the most common of the hemophilias
and is inherited as an X-linked recessive trait. Therefore males
are affected, females are carriers, and there is no male-to-male
transmission.
11

11. • If a normal male has children with a carrier of hemophilia,
there is a 50% chance that hemophilia will occur in each male
offspring and a 50% chance that each female offspring will be
a carrier.
• If a male hemophilic has children with a normal female, all
male offspring will be normal, and all female offspring will be
carriers.
12

12. 13

13. Hemophilia B
• Hemophilia B, or Christmas disease, is caused by a deficiency
of factor IX (plasma thromboplastin component) and is also
inherited as an X-linked recessive trait. Factor IX deficiency is
one fourth as prevalent as factor VIII deficiency.
• Factor XI (plasma thromboplastin antecedent) deficiency is
inherited as an autosomal recessive trait, with male and female
offspring equally affected.
14

14. Von Willebrand disease
• Its a hereditary bleeding disorder resulting from an
abnormality of the von Willebrand factor (VWF) that
is found in the plasma, platelets, megakaryocytes, and
endothelial cells. VWF circulates in conjunction with
factor VIII and is important in platelet adhesion to the
sub-endothelium via collagen.
15

15. • PROCOAGULANT CLASSIFICATION
Hemophilia A (factor VIII deficiency) and hemophilia B ( factor
IX deficiency) are classified into the following three groups
based on the level of the procoagulant present
• normal levels range from 55% to 100%
• Severe deficiency: levels less than 1%
• Moderate deficiency: levels between 1% and 5%
• Mild deficiency: levels greater than or equal to 5%
16

16. • Patients with severe deficiency may experience
 Frequent bleeding episodes, occurring two to four times
per month.
 Bleeding episodes are often spontaneous, without a
specific history of trauma.
 The common sites of bleeding include joints, muscles,
and skin.
 Hemarthroses (joint hemorrhages) are common, and
symptoms include pain, stiffness, and limited motion.
17

17. • Repeated episodes of hemarthroses or muscle hemorrhage
result in chronic musculoskeletal problems.
• Individuals with hemophilia may develop debilitating painful
arthritis. Commonly affected joints include knees, elbows,
ankles, hips, and shoulders.
• Pseudotumors (hemorrhagic pseudocysts) may occur in several
locations including the jaw, in which case curettage is
indicated.
18

18. • Patients with moderate deficiency experience less frequent
bleeding episodes (approximately four to six times per year).
• However, if a patient with moderate deficiency develops a
target joint (a joint with repeated episodes of bleeding),
spontaneous bleeding may occur.
• Patients with mild deficiency bleed infrequently and only in
association with surgery or injury.
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19. Dental management
• Use an atraumatic technique. In the event that oral surgery is
necessary, a sound surgical technique to minimize trauma and
local measures to control bleeding such as careful atraumatic
suturing and socket dressings are mandatory.
• Maxillary infiltration anaesthesia can generally be
administered slowly without pretreatment with platelet or
factor replacement..
20

20. • Avoid mandibular block injections as these may be
complicated by dissecting haematoma and airway obstruction.
In the absence of suitable factor replacement, intra-periodontal
injections may be used, but with great caution.
• The anaesthetic solution is placed under moderate pressure by
inserting the needle into the gingival sulcus and the
periodontal ligament space.
21

21. • Nitrous oxide sedation can be effective for restorative
procedures with the need for local anaesthesia; however, care
must be taken when placing matrix bands.
• Use rubber dam to protect the soft tissues.
• Sometimes, pulp exposures in primary and permanent teeth
may be avoided if carious dentin is not entirely removed in one
procedure (indirect pulp therapy). A pulpotomy or pulpectomy
is preferable to extraction. 22

22. • Periodontal treatment with deep scaling and subgingival
curettage requires factor replacement.
• Multiple extractions require hospital admission and
haematological work-up in conjunction with the haematology
team.
• Most restorative procedures on primary teeth can be
successfully completed without factor concentrate replacement
using PDL injections of local anesthesia or local infiltration.
23

23. DISEASES OF RED BLOOD CELLS
(ERYTHROCYTES):-
24

24. ANAEMIA
 At birth, haemoglobin is 20g/dl and at 3 months
lower limit of normal is taken as 9.5g/dl. A
haemoglobin level of 12g/dl or less is usually
regarded as anaemia in adult males and less than
11g/dl is taken as anaemia in females.
25

25. SYMPTOMS
 Pallor or lack of color
 Fatigue, dizziness, headaches
 Decreased exercise tolerance
 Rapid heartbeat, and shortness of breath
 Haemic murmurs
 Untreated anemia may progress to death from
heart failure 26

26. 27

27. ETIOLOGICAL
CLASSIFICATION OF ANEMIA
 Blood loss:
Acute Post hemorrhagic
Chronic blood loss
 Deficiency of Hemopoetic factors:-
Iron deficiency
Folate and vitamin b12deficiency
Protein deficiency.
 Bone marrow aplasia:-
Aplastic anemia
Pure red cell aplasia
28

28.  Anemia due to systemic infections:-
Due to chronic infection
Due to chronic renal disease
Due to chronic liver disease
Endocrinal diseases
 Anemia due to bone marrow infiltration:-
Leukemia’s
Lymphomas
Myelofibrosis
Multiple myeloma
Congenital sideroblastic anemia
 Anemia due to increased red cell destruction:-
Intra-corpuscular defect
Extra-corpuscular defect
29

29. Morphological classification of anemia:-
 Microcytic hypochromic
 Normocytic normochromic
 Macrocytic normochromic
30

30. TYPES OF ANAEMIA
 Macrocytic anemia: Megaloblastic anemia and non-
megaloblastic macrocyctic anemia. Primary cause of
this sort of anemia is collapse of DNA synthesis with
kept RNA synthesis that occurs due to the division of
the divisional cells.
31

31. • Cause: usually in children but may be associated with
celiac disease or Crohn’s disease or vegetarian with
inadequate diatery intake
32

32. • Oral manifestation:
– Angular cheilitis
– stomatitis
– Pharyngitis
– Sever glossitis
– Glossophyrosis with difficulty in swallowing
– Neurological dysfunction
33

33. • Treatment :
– Symptomatic dental treatment
– Supplementation as per individual
requirement
34

34. • Microcytic anemia: type of anemia occurs due to
hemoglobin synthesis shortage or collapse.
• Oral manifestation: painful, atrophic tongue
• Decreased healing response
• Pale mucosa
• Angular Cheilitis
• Gingivities and periodontits
• Milk bottle caries 35

35. • Treatment
– Maintenance of thorough oral hygiene,
– Do not put to sleep with bottle in mouth
– Symptomatic treatment
– Supplementation as per the requirement of
the patient
36

36.  Iron-deficiency anaemia – hypochromic microcytic
anemia characterized by low serum iron, increased
serum iron-binding capacity, decreased serum
ferritin, and decreased marrow iron stores.
38

37. Anemia of folate deficiency
• Oral manifestation: angular cheilitis
– Stomatitis
– Pharyngitis
– GIT dysfunction
• Treatment: symptomatic dental treatment
– Supplementation as per the individual requirment
39

38.  Megaloblastic (pernicious) anaemia – lack of Vit
B12
 Oral manifestation – apthous ulcer
 Hunter’s glossitis
 Beefy red colour tongue
 Management – supportive therapy by blood
transfusion
 Hydroxycobalamin 1000mg IM daily for I week
and followed by 100mg once in three months as
maintenance dose
40

39. • Hemolytic anaemia – increased rate of
erythrocyte destruction
• Management : splenectomy
– Folic acid therapy
– Prophylactic antibiotics
– Antibiotic coverage before dental treatment
especially in splenectomized children
41

40. • Sickle cell anemia – autosomal recessive anemia
characterized crescent or sickle-shaped erythrocytes
and accelerated hemolysis, due to substitution of a
single amino acid - chromosome 11
42

41. • Oral manifestation: dense lamina dura
– Hypomineralization of dentin
– Hypercementosis
– Jaw pain caused by infection
– Mental nerve palsy
– Osteoporosis of the jaw due to bone marrow
hyperplasia
43

42. • Management: Restoration of teeth under local anesthesia is
preferable to extraction.
• Non-vital primary teeth, however, should only receive a Pulpectomy
if the dentist can be reasonably confident that they can be
maintained in a non infected state; otherwise they should be
extracted.
• General anesthesia should be avoided because of the risk of crisis
induced by hypoxia; however, if it is the best alternative for
treatment, a consultation from an experienced anesthesiologist
should be taken.
44

43. β Thalassemia major
• β Thalassemia major results in a failure to produce
βglobin chains, impeding the synthesis of normal globin
chains, leading to damage of the red cell membrane,
followed by cell lysis and severe anemia.
• The body responds by increasing the production of
erythrocytes, causing increased marrow capacity in bones
and extra medullary hematopoiesis, particularly in the
spleen and liver. 45

44. • Iron overload, the main cause of morbidity and mortality, is
controlled by the iron chelating drug desferrioxamine.
• Conventional treatment related complications include post
trans fusional viral infections and multiple endocrine
dysfunction, progressive liver fibrosis, and cardiac disease due
to iron overload.
46

45. Dental Manifestations and Treatment
Concerns
• The best known oral manifestation of β thalassemia is
enlargement of the maxilla due to bony, which leads to an
increased overjet and spacing of the upper incisors.
• The maxillary sinuses may be obliterated due to erythroid
hyperplasia.
47

46. • There is generalized rarefaction of alveolar bone, thinning of
cortical bone, coarse trabeculae of the jaws, and a "chicken
wire" appearance of enlarged marrow spaces.
• The lamina dura may also be thin, and the roots of the teeth
may be short.
• Usually the mandible becomes less enlarged than the maxilla
apparently because the dense cortical plates of the mandible
prevent the expansion
48

47. • Surgical correction can be done, but relapse
can be expected because of the continued
marrow expansion.
• Lateral skull radiographs show a typical "hair
on end" calvarium.
• Pain and swelling of the parotid glands and
atrophic candidiasis have also been reported
in these patients.
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48. • Most children with the disorder can be treated normally under
local anesthesia and nitrous oxide inhalation.
• General anesthesia should be used with care in these patients
because of the cardiomyopathy, liver concerns, and endocrine
complications they present.
• A preoperative consultation with an experienced
anesthesiologist is warranted.
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49. LEUKEMIA
• Malignancy is second only to accidents as the leading cause of
death in children.
• Leukemias are hematopoietic malignancies in which there is
a proliferation of abnormal leukocytes in the bone marrow and
dissemination of these cells into the peripheral blood.
• The abnormal leukocytes (blast cells) replace normal cells in
bone marrow and accumulate in other tissues and organs of the
body.
51

50. • Leukemia is classified according to the morphology of the
predominant abnormal white blood cells in the bone marrow.
• These types are further categorized as acute or chronic, depending
on the clinical course and the degree of differentiation, or
maturation, of the predominant abnormal cells.
• In the United States, about 6550 new cases of cancer are
diagnosed each year in children under the age of 15.
52

51. Acute leukemia
• Is the most common malignancy in children, with about 2500
new cases diagnosed annually in the United States.
• Thus acute leukemia accounts for about one third of all
childhood malignancies; of these, approximately 80% are
lymphocytic (acute lymphocytic leukemia, or ALL)
53

52. Chronic leukemia
• in children is rare, accounting for less than 2% of all cases.
And the most common is chronic lymphocytic leukemia (CLL)
• Leukemia affects about one of every 29,000 children each
year in the United States.
• The peak incidence is between 2 and 5 years of age.
54

53. • Although the cause of leukemia is unknown, ionizing
radiation, certain chemical agents, and genetic factors have
been implicated.
• For example, children with chromosomal abnormalities
(Down syndrome and Bloom syndrome), children with an
identical twin who has leukemia, and children with
immunologic disorders have an increased risk for leukemia.
55

54. Some of the more common findings are
• Pallor
• Fever
• Tachycardia
• Adenopathy
• Hepatosplenomegaly
• Petechiae
• Cutaneous bruises
• Gingival bleeding
• Evidence of infection.
56

55. ORAL MANIFESTATIONS OF
LEUKEMIA
• Pathologic changes in the oral cavity as a result of leukemia occur frequently.
The most frequently reported oral abnormalities attributed to the leukemic process
include
• Regional lymphadenopathy
• Mucous membrane petechia
• Ecchymoses
• Gingival bleeding
• Gingival hypertrophy
• Pallor,
• Nonspecific ulcerations. 57

56. • Manifestations seen occasionally are
• Cranial nerve palsies
• Chin and lip paresthesias
• Odontalgia,
• Jaw pain
• Loose teeth, extruded teeth,
• gangrenous stomatitis.
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57. • Each of these findings has been reported in all types of
leukemia.
• Regional lymphadenopathy is the most frequently reported
finding.
• Gingival abnormalities, including hypertrophy and bleeding,
are more common in patients with AML, whereas petechiae
and ecchymoses are more common in those with ALL.
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58. • Like the systemic manifestations of leukemia, oral changes
can be attributed to anemia, granulocytopenia, and
thrombocytopenia, all of which result from the replacement of
normal bone marrow elements by undifferentiated blast cells,
or to direct invasion of tissue by these leukemic cells.
60

59. DENTAL MANAGEMENT OF
PATIENTS WITH LEUKEMIA
• Before any dental treatment is administered to a child with
leukemia, the child’s hematologist/oncologist or primary care
physician should be consulted. The following information
should be ascertained:
1. Primary medical diagnosis
2. Anticipated clinical course and prognosis
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60. 3. Present and future therapeutic modalities
4. Present general state of health
5. Present hematologic status
• It is also important to establish, by consultation with the
patient’s physician, when dental treatment may be most
propitious, and to schedule the patient’s treatment accordingly.
62

61. • For a child whose first remission has not yet been obtained, or
one who is in relapse, all elective dental procedures should not
be done.
• However, it is essential that potential sources of systemic
infection within the oral cavity be controlled or eradicated
whenever they are recognized (e.g., immediate extraction of
carious primary teeth with pulpal involvement).
63

62. • Routine preventive, restorative, and surgical procedures can
usually be provided for a patient who is in complete remission
yet is undergoing chemotherapy.
• Pulp therapy on primary teeth is contraindicated in any patient
with a history of leukemia.
• Endodontic treatment for permanent teeth is not
recommended for any patient with leukemia who may have a
chronic, intermittent suppression of granulocytes. Even with
the most exacting technique, an area of chronic inflammatory
tissue may remain in the periapical region of endodontically
treated teeth.
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63. • An area of low-grade, chronic inflammation in a healthy
patient is generally well tolerated, but in an
immunosuppressed, neutropenic patient the same area can act
as an anchoretic focus with devastating sequelae.
• A patient who has been in complete remission for at least 2
years and no longer requires chemotherapy may be treated in
an essentially normal manner.
65

64. • A platelet level of 100,000/mm3 is adequate for most dental
procedures.
Routine preventive and restorative treatment, including local
infiltration, may be considered when the platelet count is at least
50,000/mm.
• If the platelet count is lower than 20,000/mm3, all the intraoral
mucosal tissues may show clinical evidence of spontaneous
hemorrhaging (e.g., petechiae, ecchymoses, or frank
hemorrhage). 66

65. • No dental treatment should be performed at such a time
without a preceding prophylactic platelet transfusion.
• The use of a soft nylon toothbrush for the removal of plaque is
recommended, even if the patient is thrombocytopenic.
• • As long as the gingiva remains in a healthy state and its
manipulation by brushing does not induce significant
hemorrhage.
• Scaling and subgingival curettage should not necessarily be
perceived as elective dental treatment in all patients.
67

66. • Candidiasis is common in children with leukemia.
• The following topical use of nystatin can be particularly
beneficial:
• Nystatin oral suspension, 100,000 U/mL • Swish 5 mL for 5
minutes and then swallow
• Repeat every 6 hours; continue for 48 hours after lesions
disappear
68