Bleeding disorders

Presented by
JANANI RG
 CONTENTS

HAEMOSTASIS
HAEMOPHILIAS
LEUKEMIAS
SICKLE CELL ANEMIA
CONCLUSION
REFERENCES
 COMPONENTS

Blood:
Plasma
water, albumin, electrolytes, clotting factors
Cellular Components
RBCs, WBCs, Platelets
All formed in the red bone marrow (after birth)In
utero- spleen, thymus, liver
lymphatic system regulates maturation
 MECHANISM OF HAEMOSTASIS
X
Hemophilia A
Factor VIII concentrate is used for treatment of
hemophilia A.
For mild factor VIIIdeficient hemophilia, DDAVP (1-
deamino- 8-D-arginine vasopressin) may be used for
minor hemorrhagic episodes to achieve hemostasis.
DDAVP (desmopressin acetate) is a synthetic analogue
of the natural pituitary hormone 8-arginine
vasopressin (antidiuretic hormone) affecting renal
water conservation.
This drug, when given intravenously, subcutaneously,
or intranasally causes a rise in factor VIII activity and
VWF through release from stored sites in endothelial
cells.
Hemophilia B
Factor IXdeficient hemophilia is treated with purified
coagulation factor IX concentrate.

The volume of distribution of recombinant factor IX is greater

than that of plasma-derived factor IX (estimated minimum
volume of 1.2 compared with 1, respectively, whereas in infants
and young children a minimum volume of distribution of 1.4
should be used for dose calculation).

Treatment regimens- primary & secondary

Schedule dental evaluations and interventions on regularly

planned infusion days.
Arthritis and Degenerative joint disease secondary to recurrent bleeding.
 Antifibrinolytic agents

In children, Aminocaproic acid is given immediately before

dental treatment in an initial loading dose of 100 to 200 mg/kg
by mouth up to a maximum total dose of 10 g.

Subsequently, 50 to 100 mg/kg per dose up to a total

maximum dose of 5 g is administered orally every 6 hours for 5
to 7 days.

Alternatively, for patients of approximately adult size or heavier

than 30 kg, a regimen of 3 g by mouth four times daily without
a loading dose may be used.

The adult and pediatric dosage of Tranexamic acid is 25 mg/kg

given immediately before dental treatment.
The same dose is continued every 8 hours for 5 to 7 days.
Local Anesthesia

Local anesthesia

Absence of factor replacement, PDL injections may be used.

 Periodontal management
Periodontal Therapy

Patients who require deep scaling initially undergo supragingival

For simple extractions of erupted permanent teeth and
After extractions direct topical application of haemostatic agents, such
 Endodontic Therapy:
Avoid incrementing
High-speed through
vacuum and saliva the tooth
ejectors mustapex.
be used with
caution so that sublingual hematomas do not occur.

Care must also be used in the placement of intraoral

radiographic films, particularly in highly vascular sublingual
tissues.

The preparation of a tooth for a cast crown requires caution in

gingival preparation, as does placement of retraction cord
and impression material.

Periphery wax is used on the impression tray to prevent

possible intraoral laceration during tray placement.
 Acute

The clinical manifestations of acute leukemia are

caused by the infiltration of leukemia cells into tissues
and organs.
Infiltration and proliferation of leukemia cells in the
bone marrow lead to anemia, thrombocytopenia, and
granulocytopenia.
The history at presentation may reveal increased
irritability, lethargy, persistent fever, vague bone pain,
and easy bruising
ORAL MANIFESTATIONS OF LEUKEMIA

Oral signs or symptoms suggestive of leukemia have

been reported in as many as 75% of adults and 29%
of children with leukemia.
The lower incidence of oral manifestations in
children can be attributed in part to the early age at
diagnosis and the high percentage of ALL in the
pediatric age group.
The incidence of ALL peaks at 3 years of age.
Abnormalities in or around the oral cavity occur in
all types of leukemia, and in all age groups. .
 Regional lymphadenopathy,
mucous membrane petechiae and
ecchymoses,
gingival bleeding,
gingival hypertrophy,
pallor, and nonspecific ulcerations.

Manifestations seen occasionally are cranial

nerve palsies, chin and lip paresthesias,
odontalgia, jaw pain, loose teeth, extruded
teeth, and gangrenous stomatitis.
 Very high circulating white blood cell numbers in the
peripheral blood can lead to stasis in small vascular
channels.
tissue anoxia results in areas of necrosis and
ulceration that can readily become infected by
opportunistic oral microorganisms in patients with
neutropenia.

Infiltration of leukemic cells along vascular channels

can result in strangulation of pulpal tissue
 The teeth may rapidly loosen as a result of necrosis of
the periodontal ligament..
The most common finding is a generalized osteoporosis
caused by enlargement of the Haversian canals and
Volkmann canals.
Osteolytic lesions resulting from focal areas of
hemorrhage and necrosis and leading to loss of
trabecular bone are also common.
Manifestations in the jaws include generalized
loss of trabeculation,
destruction of the crypts of developing teeth,
loss of lamina dura,
widening of the periodontal ligament space, and
displacement of teeth and tooth bud.
DENTAL MANAGEMENT OF PATIENTS WITH
Before any dental treatment is administered
LEUKEMIA to a child
with leukemia, the childs 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
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
patients physician, when dental treatment may be most
propitious, and to schedule the patients treatment
accordingly.
 Before the appointment preferably the same daya
blood cell profile
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.
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.
X

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, it is not
appropriate to discontinue the use of a
toothbrush because of the platelet level alone
Scaling and subgingival curettage should not
necessarily be perceived as elective dental
treatment in all patients.
 Use of topical obtundents for pain may be the only treatment
indicated.
Local measures, such as the topical application of either
bovine thrombin or Avitene, and the placement of an oral
adhesive for protection, may be benefi cial.
In a patient who is physically debilitated or who is in relapse,
septic, and severely granulocytopenic, ulcerative lesions
require close observation.
Such lesions may serve as a nidus for the proliferation of
microorganisms, which can lead to potentially fatal viral,
fungal, or bacterial infection.
Therefore specimens from these ulcerative lesions should be
cultured and subsequent sensitivity testing performed, and
antibiotic therapy should be initiated or modifi ed accordingly.
If the ANC is less than 1000/mm3, elective dental
treatment should be deferred.

A leukemic patient with a low ANC may require

prophylactic broad-spectrum antibiotic therapy before
certain dental procedures.

The patients physician should be consulted regarding

the appropriate drugs and dosages.

Infection and hemorrhage are the primary causes of

death other than resistant disease or relapse in
children with leukemia.
SICKLE CELL ANEMIA
Sickle cell anaemia
Abnormal hemoglobin where valine is substituted for
glutamic acid on the sixth position of the beta chain
of hemoglobin in the homozygous sickle cell
individual.

The red blood cells assume unusual shapes,

becoming more rigid and making it more difficult for
them to pass through small blood vessels, which
results in increased blood viscosity and tissue hypoxia
SICKLE CELL ANEMIA
Patients with sickle cell anemia have an autosomal
recessive hemolytic disorder that occurs
predominantly in persons of African descent but is
also found among Italian, Arabian, Greek, and Indian
people.
Patients with sickle cell anemia produce hemoglobin S
instead of the normal hemoglobin A.
Hemoglobin S has a decreased oxygen-carrying
capacity.
Decreased oxygen tension causes the sickling of cells.
Over time there is a progressive deterioration of
cardiac, pulmonary, and renal function.
Oral and Dental Considerations in Management of Sickle Cell
Anemia- Sonu Acharya. IJCPD
 Dental appointments should be short to reduce
potential stress on the patient. .
Dental treatment should not be initiated during a
sickle cell crisis.
Patients with sickle cell anemia may have skeletal
changes that make orthodontic treatment beneficial.
Special care must be taken to avoid tissue irritation,
which may induce bacteremia's, and the disease
process may compromise the proposed treatment.
Careful monitoring is a necessity when proposing
elective orthodontic treatment in patients with sickle
cell anemia.
 Most patients with sickle cell anemia are taking low-dose daily
prophylactic antibiotics, and the need for additional antibiotics
for dental procedures is debatable.
The use of nitrous oxide is not contraindicated in these
patients.
Care must be taken in treating patients with sickle cell anemia
to avoid diffusion hypoxia at the completion of the dental
procedure.
The restoration of teeth, including pulpotomies, is preferable to
extraction.
Pulpectomy in a nonvital tooth is reasonable if the practitioner
is fairly confident that the tooth can remain noninfected. If the
tooth is likely to persist as a focus of infection, then extraction
is indicated.
 The goal of the transfusion is to increase the patients
hemoglobin level to higher than 10 g/dL and to
decrease the hemoglobin S level below 40%.
Patients with a hemoglobin level of less than 7 g/dL
and a hematocrit of less than 20% may require a
transfusion.
Pediatric patients are usually less likely to have post-
transfusion complications than are adults.
A high frequency of hospitalizations is indicative of a
more severe anemia, and such patients may require
transfusion before surgery.
Minor surgeries may not require a transfusion.
 CONCLUSION

Oral manifestations are important findings

in bleeding disorders. Understanding the
mechanism helps identify potential illness
at an early stage
 REFERENCES
Mc donald and avery. Dentistry for the child and
adolesent . 9th edition
Shafers Oral Pathology, 6th edition
FranchAM, Gavalda Esteve CG, Perez MS, Oral
manifestations and dental management of patient with
leukocyte alterationsJ Clin Exp Dent. 2011;3(1):e53-9.
Israels S Boyar R, Bleeding Disorders: Characterization,
Dental Considerations and Management J Can Dent
Assoc 2006; 72(9):827
Derossi SS ,Garfunkel A, Greenberg MS ,Hematologic
Diseases. Burkets Oral Medicine and Diagnosis, tenth
edition.