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Dental management of
patients with bleeding
disorders
Supervised by Prof. Hafez Diab
Written and Collected by
5th stage students:

By:
Saja Khairi Farhan

26.June.2020

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Introduction
Bleeding disorders are a group of disorders that share the inability to form a proper
blood clot, they are characterized by extended bleeding after injury, surgery, trauma or
menstruation. Sometimes the bleeding is spontaneous, without a known or identifiable
cause, improper clotting can be caused by defects in blood components such as platelets
and/or clotting proteins, also called clotting factors. The body produces 13 clotting
factors, if any of them are defective or deficient, blood clotting is affected; a mild,
moderate or severe bleeding disorder can result.

Some bleeding disorders, such as hemophilia, can be inherited or acquired. Others can
occur from such conditions as anemia, cirrhosis of the liver, HIV, leukemia and vitamin
K deficiency. They also can result from certain medications that thin the blood, including
aspirin, heparin and warfarin

Classification of Bleeding Disorders

I. Nonthrombocytopenic purpuras

a. Vascular wall alterations (1) Scurvy (2) Infection (3) Chemicals (4) Allergy

b. Disorders of platelet function:

1- Genetic defects (Bernard-Soulier disease).

2- Drugs (a) Aspirin (b) NSAIDs (c) Alcohol (d) Beta-lactam antibiotics (e) Penicillin (f)
Cephalothins

3- Allergy

4- Autoimmune disease

5- Von Willebrand's disease (secondary factor VIII deficiency).

6- Uremia.

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II. Thrombocytopenic purpuras

a. Primary—idiopathic.

b. Secondary:

(1)Chemicals

(2) Physical agents (radiation)

(3) Systemic disease (leukemia)

(4) Metastatic cancer to bone

(5) Splenomegaly

(6) Drugs: (a) Alcohol (b) Thiazide diuretics (c) Estrogens (d) Gold salts

(7)Vasculitis

(8)Mechanical prosthetic heart valves

(9)Viral or bacterial infections

III. Disorders of coagulation

a. Inherited

(1- Hemophilia A (deficiency of factor VIII

2-Hemophilia B (deficiency of factor IX)

3- Others

b. Acquired

1-Liver disease

2-Vitamin deficiency (a) Biliary tract obstruction (b) Malabsorption (c) Excessive use of
broad-spectrum antibiotics

3-Anticoagulation drugs (a) Heparin (b) Coumarin (c) Aspirin and NSAIDs

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4-Disseminated intravascular coagulation (DIC).

5-Primary fibrinogenolysi.

Some of inherited bleeding disorders

Vascular wall defects

A patient’s bleeding disorder may be unrecognized, and bleeding episodes can appear
spontaneously or after dental extraction. Patients with inherited bleeding disorders that
are marked by a vascular defect, such as Marfan syndrome, hereditary hemorrhagic
telangiectasia (Osler-Weber-Rendu syndrome), and Ehlers-Danlos syndrome fig(1),
rarely have a severe bleeding episode after dental extraction. Usually, these patients can
receive dental treatment in the primary care setting. Management includes coordination
with the hematologist, minimally invasive dentistry, local hemostatic measures, and
avoiding analgesics such as aspirin and other nonsteroidal anti-inflammatory drugs.

Fig(1) Ehlers-danlos syndrome

Platelet defects

Inherited platelet defects result in qualitative dysfunction, and patients are best treated
after consultation with a hematologist, who might advocate for platelet transfusion prior
to any invasive dental procedures.

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Hemophilia A

Hemophilia A is an inherited coagulation disorder involving a deficiency of factor

VIII.1 The prevalence of hemophilia A is different from country to country and depends
on the national economies. For example, countries with a high income have the highest
prevalence, which increases over time.

Hemophilia A is inherited as an autosomal X-linked recessive trait; therefore, the

disease mainly affects males.

However, females with a factor VIII activity level of less than 50% are considered to be
carriers and are treated as patients with mild hemophilia.

Von Willebrand disease

is an inherited disease marked by vWF deficiency. It is considered the most common

congenital bleeding disorder, affecting 1% of the population of both sexes equally;
symptomatic prevalences are reported to range from 1 in 1000 to 1 in 10,000 of the
population. In rare cases, acquired von Willebrand disease may develop in elderly
patients and is associated with various underlying diseases.

Von Willebrand factor acts as a carrier for factor VIII and increases its half-life, In
addition, vWF attaches to collagen and membranes of blood vessels. All these functions
aid in clot formation.

Three types of vWD with different subtypes and different patterns of inheritance have
been recognized: type 1(mild), type 2 (moderate), and type 3 (severe).

This classification is important to help understand the dental management and reduce
the risk of bleeding following dental procedures. The clinical features of this disease
range from an unrecognized, mild form that requires local hemostatic measures to a
severely debilitating form that requires preoperative and postoperative hematologic
prophylaxis.

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fig(2) vWD in children

Diagnostic Considerations
Clinical Symptoms
Bleeding is a common symptom and does not always indicate an underlying bleeding
disorder. Symptoms such as gum bleeding, epistaxis, menorrhagia, petechiae, and
bruising are especially common; in one study they were reported by anywhere from 22%
to 85% of men and women without bleeding disorders, Identification of pathologic
bleeding may, therefore, prove challenging.

Symptoms of a bleeding disorder include :

1-Bleeding into joints, muscles and soft tissues.

2-Excessive bruising.

3-Prolonged, heavy menstrual periods (menorrhagia ).

4-Unexplained nosebleeds.

5-Extended bleeding after minor cuts, blood draws or vaccinations, minor surgery or
dental procedures.

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Clinically significant mucocutaneous bleeding is defiend as any of the following ;

spontaneous or provoked bleeding from 2 or more distinct mucocutaneous sites; bleeding
from a single site warranting blood transfusions; or bleeding from a single site on 3 or
more separate occasions.

Bleeding scoring systems have shown promise in retrospectively predicting bleeding

phenotype in type 1 VWD5 and prospectively excluding mild bleeding disorders in
patients presenting with bleeding symptoms or abnormal coagulation study results, but
they require further investigation and validation for broader clinical use.

History and Physical Examination:

Thorough history and physical examination often provides clues as to whether
bleeding is pathologic and may even point to potential underlying causes.

During physical examination, the skin and mucous membranes should be inspected
for stigmata of bleeding (eg, bruising, petechiae) and other findings suggestive of
potential underlying causes of bleeding (eg, jaundice, telangiectasia).

The presence of hepatomegaly, splenomegaly, or joint hypermobility may suggest

potential diagnoses associated with bleeding. Skin or conjunctival pallor, tachycardia, or
a cardiac flow murmur may indicate associated anemia. Historical and physical findings
may suggest an abnormality of either (a)primary hemostasis, which culminates in the
formation of a platelet plug;(b) secondary hemostasis, in which fibrin is formed via the
coagulation “cascade”; or fibrinolysis, the normal breakdown of clots.

The laboratory Investigations

The laboratory evaluation for bleeding includes conducting initial screening tests. The
most common screening tests that can be performed in the emergency room are:

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1-PT—Activated by tissue thromboplastin.

a.Tests extrinsic and common pathways.

b. Control should be run.

c.Normal (11-15 seconds, depending on laboratory).

d. Control must be within normal range.

2-aPTT—Initiated by phospholipid platelet substitute and activated by addition of

contact. activator (kaolin).

a. Tests intrinsic and common pathways

b. Control should be run

c. Normal (25-35 seconds, depending on laboratory)

d. Control must be within normal range

3-TT—Activated by thrombin
a. Tests ability to form initial clot from fibrinogen

b. Controls should be run

c. Normal (9-13 seconds.

4-PFA-100
a. Tests platelet function

b. Normal if adequate number of platelets of good quality present

c. Normal (< 175 seconds)

5-Platelet acount
a. Tests platelet phase for adequate number of platelets

b. Normal (140,000-400,000/mm 3 )

c. Clinical bleeding problem can occur if less than 50,000/mm 3

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Thrombin time (TT) is of novel interest to emergency physicians as it helps them assess
the bleeding tendency in patients using new anticoagulants. The TT is the time required
to convert fibrinogen into a fibrin clot, bypassing the intrinsic, extrinsic and common
pathways.

For an individual with known hemophilia, routine laboratory investigations (PT, PTT,
factor levels) are not indicated for a routine bleeding episode unless requested by the
patient’s hematologist.

The clinical severity of a patient’s hemophilia is gauged by his or her baseline clotting
factor level, a value that remains constant throughout that person’s life.

Alternatively, an individual with an undiagnosed bleeding disorder may present with

abnormal haematologic laboratory results obtained as a part of routine evaluation or an
evaluation for surgery or for some other reason.

A prolongation of aPTT or PT of a patient who is not on any prescribed anticoagulants

may indicate an acquired or congenital clotting factor deficiency or an inhibitor of one or
more coagulation factor.

Bleeding Disorder Treatments

There are three general treatments for bleeding disorders:

Risk Reduction
Patients with bleeding disorders should avoid medications that thin the blood. They
may also need to make some changes to lifestyle or activities to reduce their risk of
bleeding. For patients who require surgery, physicians can take steps to reduce the risk of
operative and post-operative bleeding. Learn more about blood disorder surgical
concerns .

Medications

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Several drugs are available that improve blood coagulation or help prevent clots from
dissolving. physician will develop a treatment plan tailored to individual disease and
health needs.

Replacement Therapy
Patients with moderate to severe bleeding disorders may require transfusion of blood
platelets or clotting factors. Clotting factors may be donated human blood products or
lab-synthesized proteins. Patients with severe bleeding disorders may receive clotting
factor transfusions as a preventive measure

For dental consideration dentists must be aware of the impact of bleeding disorders on
the management of their patients. Proper dental and medical evaluation of patients is
therefore necessary before treatment, especially if an invasive dental procedure is
planned. Patient evaluation and history should begin with standard medical
questionnaires. Patients should be queried about any previous unusual bleeding episode
after surgery or injury, spontaneous bleeding and easy or frequent bruising .

Dental Management
The management of patients with bleeding disorders depends on the severity of the
condition and the invasiveness of the planned dental procedure. If the procedure has
limited invasiveness and the patient has a mild bleeding disorder, only slight or no
modification will be required. In patients with severe bleeding disorders, the goal is to
minimize the challenge to the patient by restoring the hemostatic system to acceptable
levels and maintaining hemostasis by local and adjunctive methods. The patient’s
physician should be consulted before invasive treatment is undertaken. In patients with
drug-induced coagulopathies, drugs may be stopped or the doses modified. For
irreversible coagulopathies, replacement of missing factors may be necessary .

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1-Pain Control In patients with coagulopathies

nerve-block anesthetic injections are contraindicated unless there is no better
alternative and prophylaxis is provided, as the anesthetic solution is deposited in a highly
vascularized area, which carries a risk of hematoma formation. The commonly used
blocks require minimum clotting factor levels of 20% to 30%. Extravasation of blood in
the oropharyngeal area by an inferior alveolar block or in the pterygoid plexus can
produce gross swelling, pain, dysphasia, respiratory obstruction and risk of death from
asphyxia. Anesthetic infiltration and intraligamentary anesthesia are potential
alternatives to nerve block in many cases. An anesthetic with a vasoconstrictor should be
used when possible. Alternative techniques, including sedation with diazepam or nitrous
oxide–oxygen analgesia, can be employed to reduce or eliminate the need for anesthesia.
Patients undergoing extensive treatment requiring factor replacement may be treated
under general anesthesia in a hospital operating room.

2-Dental extraction
(a)Mild form of disease After consultation with the hematologist prior to dental
extraction, patients with the mild forms of hemophilia A and vWD are normally treated
preoperatively by desmopressin acetate, which stimulates the release of vWF from the
Weibel-Palade bodies of endothelial cells.

This in turn increases the levels of vWF (as well as coagulant factor VIII) threefold to
fivefold. One hour prior to the dental procedures, desmopressin acetate can be
administered intravenously (0.3 µg/kg in 50 mL of normal saline), subcutaneously (0.3
µg/kg using the 15- µg/mL concentration), or intranasally (150 µg).

However, intravenous administration of desmopressin acetate may have cardiovascular

side effects, such as a slightly elevated heart rate, hypotension, and headache.
Desmopressin acetate is not recommended for young children and patients with ischemic
heart disease .

(b)Moderate to severe forms of disease

Before dental extractions, patients with moderate to severe disease require replacement
therapy with either factor concentrate (patients with either disease) or recombinant factor
VIII (patients with hemophilia A); there is no recombinant therapy available for vWF

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However, factor concentrate is expensive and may result in the development of

autoantibodies or inhibitors that impair clotting, which is why unnecessary dental
procedures should be avoided. This will help to decrease the repetition of replacement
therapy and reduce the chance of inhibitor formation. Replacement therapy can be
administered by the healthcare provider, the patient’s caregiver, or the patient as a
prophylactic option and/or as emergency treatment in case of prolonged bleeding.

It is recommended to deliver the intended dental treatment within 30-60 minutes

following the administration of factor concentrate. Recombinant factor VIII can be given
in patients with the moderate to severe forms of hemophilia A, which helps reduce the
possibility of blood-borne infection that could result from transfusion of infected blood.

3-Oral Surgery
Surgical procedures carry the highest risk of bleeding, and safety precautions are needed.
For coagulopathies, transfusion of appropriate factors to 50% to 100% of normal levels is
recommended when a single bolus infusion is used in an outpatient setting. In patients
with hemophilia, additional postoperative factor maintenance may be required after
extensive surgeries. This can be done with factor infusion, DDAVP, cryoprecipitate or
fresh frozen plasma depending on the patient’s condition. The patient’s hematologist
should be consulted before planning, and patients with severe disease should be treated in
specialty centres.

Local hemostatic agents (Table 1) and techniques such as pressure, surgical packs,
sutures and surgical stents may be used individually or in combination and may assist in
the local delivery of hemostatic agents, such as topical thrombin and vasoconstrictors.

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Table(1)

However caution is needed with the use of vasoconstrictors because of the risk of
rebound vasodilatation, which may increase late bleeding risk. The use of absorbable
hemostatic materials may favour clot formation and stability.

However, these materials also carry a risk of infection and may delay healing; they
should therefore be avoided in immunosuppressed patients.

Topical thrombin is an effective agent when applied directly on the bleeding wound as
it converts fibrinogen to fibrin and allows rapid hemostasis in a wound. Topical fibrin
glue can reduce the amount of factor replacement needed when used along with
antifibrinolytic agents.

Fibrin glue has also been effectively used in conjunction with other hemostatic
measures.

The use of drugs affecting bleeding mechanisms does not usually pose a significant
problem in dental treatment. If ASA has to be withdrawn, this should be done at least 10
days before surgery.

In most cases, ASA therapy does not need to be stopped, and local hemostatic
measures are sufficient to control bleeding. Similarly, other antiplatelet drugs, such as
clopidogrel and dipyridamole, usually do not need to be stopped.

The patient’s physician should be consulted before any decision is made to modify the
patient’s drug regimen, and the potential risk–benefit ratio should be determined

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4-Periodontal Procedures
Periodontal health is of critical importance in patients with bleeding disorders3 as
inflamed and hyperemic gingival tissues are at increased risk of bleeding. Periodontitis
may cause tooth mobility and warrant extraction, which may be a complicated procedure
in these patients. Patients with coagulopathies may neglect their oral health due to fear of
bleeding during tooth brushing and flossing, which leads to increased gingivitis,
periodontitis and caries.

Periodontal probing, supragingival scaling and polishing can be done normally without
the risk of significant bleeding. Factor replacement is seldom needed for subgingival
scaling and root planing if these procedures are done carefully .

Ultrasonic instrumentation may result in less tissue trauma.

For severely inflamed tissues, initial treatment with chlorhexidine mouthwashes and
gross debridement is recommended to reduce tissue inflammation before deep scaling

Factor replacement may be required before extensive periodontal surgery and use of
nerve blocks. Periodontal packing materials and custom vinyl mouthguards (stents) are
used to aid in hemostasis and protect the surgical site, but these can be dislodged by
severe hemorrhage or subperiosteal hematoma formation. Antifibrinolytic agents may be
incorporated into periodontal dressings for enhanced effect. Post-treatment
antifibrinolytic mouthwashes are usually effective in controlling protracted bleeding.

5-Restorative and Endodontic Procedures

General restorative procedures do not pose a significant risk of bleeding. Care should be
taken to avoid injuring the gingiva while placing rubber dam clamps, matrices and
wedges. A rubber dam should be used to prevent laceration of soft tissues by the cutting
instruments. Saliva ejectors and high-speed suction can injure the mucosa in the floor of
the mouth and cause hematoma or ecchymosis; thus, they should be used carefully.
Endodontic therapy is preferred over extraction whenever possible in these patients.
Endodontic therapy does not usually pose any significant risk of bleeding and can be
performed routinely. Endodontic surgical procedures may require factor replacement
therapy.
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6-Prosthodontic Procedures
These procedures do not usually involve a considerable risk of bleeding. Trauma should
be minimized by careful post-insertion adjustments. Oral tissue should be handled
delicately during the various clinical stages of prosthesis fabrication to reduce the risk of
ecchymosis. Careful adjustment of prostheses is needed to reduce trauma to soft tissue.

7-Orthodontic Procedures
Orthodontic therapy can be carried out without bleeding complications, although care
should be taken that appliances do not impinge on soft tissues and emphasis should be
put on excellent, atraumatic oral hygiene

8-Choice of Medications
Many medications prescribed in dental practice especially ASA, may interfere with
hemostasis.

In addition, many drugs interact with anticoagulants, increasing their potency and the
risk of bleeding.

When used for prolonged periods, ASA and nonsteroidal antiinflammatory drugs
(NSAIDS) can increase the effect of warfarin.

Penicillins, erythromycin, metronidazole, tetracyclines and miconazole also have

potentiating effects on warfarin.

Care should be taken when prescribing these drugs to patients with bleeding tendencies
or those receiving anticoagulant therapy, and it may be desirable to consult the patient’s
physician before planning the dose regimen .

Review for Dental Management

Conclusion Patients with mild bleeding disorders can be treated in a primary care
setting after consultation with the hematologist, while patients with a moderate to severe
level of bleeding disorder who require invasive dental procedures are best treated in a
hospital setting.

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Consultation with the hematologist prior to any dental procedure is recommended to

assess the patient’s needs for prophylactic replacement therapy.

Factor replacement therapy is required before inferior alveolar nerve block, lingual
infiltration, or floor of the mouth injection.

Restorative, prosthodontic, endodontic, and orthodontic treatments are considered safe

in the majority of patients with bleeding disorders unless more complex dentistry is
required.

The use of aspirin and other nonsteroidal anti-inflammatory drugs (such as ibuprofen
and naproxen sodium) should be avoided in patients with bleeding disorders.

Dental care providers must immediately report cases of prolonged bleeding, dysphagia,
or difficulty speaking and breathing following dental procedures to the patient’s
hematologist.

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References
1-Bleeding Disorders of Importance in Dental Care and Related Patient Management
Anurag Gupta, BDS; Joel B. Epstein, DMD, MSD, FRCD(C); Robert J. Cabay, MD,
DDS.

2-Pototski M, Amen fibar JM. Dental management of patients receiving anticoagulation

or antiplatelet treatment. J Oral Sci. 2007 p253-258.

3-Identification and Basic Management of Bleeding Disorders in Adults Rebecca Kruse-

Jarres, MD, Tammuella C. Singleton, MD, and Cindy A. Leissinger, MD.

4-Dental management of patients with inherited bleeding disorders: a multidisciplinary

approach Hassan Abed, BDS, MSc Abdalrahman Ainousa, BDS, MSc.

5-Little: Dental Management of the Medically Compromised Patient, 7th ed.

6-Approach to Patients with Bleeding Disorders in the Emergency Department Hanan

Alhajri, Maryam Darwish, Devendra Richhariya and Ayesha Almemari* Emergency
Department, Mafraq Hospital – Seha, United Arab Emirate.

7-Irwin R. Walker, MB, BS, FRCPC The Bleeding Disorders: Current Concepts and
Management.

8-Webster WP, Roberts HR, Penick GD. Dental care of patients with hereditary disorders
of blood coagulation. Mod Treat. 1968;p :93-110.

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