Pratique Clinique

Bleeding Disorders of Importance in Dental Care

and Related Patient Management
Auteur-ressource
Anurag Gupta, BDS; Joel B. Epstein, DMD, MSD, FRCD(C); Robert J. Cabay, MD, DDS Dr Epstein
Courriel : jepstein@
uic.edu

SOMMAIRE

Les fournisseurs de soins buccodentaires doivent être conscients de l’impact des

troubles du saignement sur la prise en charge des patients dentaires. La découverte
initiale d’un trouble du saignement, pouvant indiquer la présence d’un processus
pathologique général, peut avoir lieu au sein du cabinet dentaire. En outre, il est pré-
férable que les soins dentaires préventifs, restaurateurs et chirurgicaux soient accomplis
par des praticiens compétents en ce qui concerne la pathologie, les complications et les
options de traitement associées à celle-ci. Cet article examine les troubles du saignement
communs et leur effet sur la prestation de soins de santé buccodentaire.

Pour les citations, la version définitive de

Mots clés MeSH : blood coagulation/physiology; blood coagulation disorders/complications; dental care cet article est la version électronique :
www.cda-adc.ca/jcda/vol-73/issue-1/77.html

D
entists must be aware of the impact of emergency department and do not affect dental
bleeding disorders on the management of treatment significantly.
their patients. Proper dental and medical The patient should be asked for any history of
evaluation of patients is therefore necessary be- significant and prolonged bleeding after dental
fore treatment, especially if an invasive dental extraction or bleeding from gingivae. A history
procedure is planned. Patient evaluation and of nasal or oral bleeding should be noted. Many
history should begin with standard medical bleeding disorders, such as hemophilia and von
questionnaires. Patients should be queried about Willebrand’s disease, run in families; therefore,
any previous unusual bleeding episode after sur- a family history of bleeding disorders should be
gery or injury, spontaneous bleeding and easy carefully elicited.
or frequent bruising. For the purpose of history- A complete drug history is important. If a
taking, a clinically significant bleeding episode1 patient is taking anticoagulant drugs, it will be
is one that:
important to consult his or her physician before
• continues beyond 12 hours any major surgical procedure. In addition, a
• causes the patient to call or return to the number of medications may interfere with he-
dental practitioner or to seek medical treat- mostasis and prolong bleeding. Drugs of abuse,
ment or emergency care such as alcohol or heroin, may also cause excess
• results in the development of hematoma or bleeding 2 by causing liver damage resulting in
ecchymosis within the soft tissues or altered production of coagulation factors. Illicit
• requires blood product support. injection drug use carries an increased risk of
Most reported bleeding episodes are minor transmission of viral pathogens that may lead to
and do not require a visit to the dentist or the viral hepatitis and altered liver function.

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Table 1 Common bleeding disorders • bleeding time to determine platelet func-

tion (normal range: 2–7 minutes)
Coagulation factor Congenital
• activated partial thromboplastin time to
deficiencies Hemophilia A and B
evaluate the intrinsic coagulation pathway
von Willebrand’s disease
(normal range: 25 ± 10 seconds)
Other factor deficiencies (rare)
• international normalized ratio to measure
Acquired
the extrinsic pathway (normal range: 1.0)
Liver disease
• platelet count to quantify platelet function
Vitamin K deficiency, warfarin use
(normal range: 150,000–450,000/µL).
Disseminated intravascular coagulation
Platelet disorders Quantitative disorder (thrombocytopenia) Types of Bleeding Disorders
Immune-mediated Bleeding disorders can be classified as
Idiopathic coagulation factor deficiencies, platelet disor-
Drug-induced ders, vascular disorders or fibrinolytic defects
Collagen vascular disease (Table 1). 3,4
Sarcoidosis Among the congenital coagulation de-
Non-immune-mediated fects, hemophilia A, hemophilia B (Christmas
Disseminated intravascular coagulation disease) and von Willebrand’s disease are
Microangiopathic hemolytic anemia the most common. Hemophilia A is due to a
Leukemia deficiency of clotting factor VIII or antihe-
Myelofibrosis mophilic factor. It is an inherited X-linked
Qualitative disorder recessive trait found in males. Symptoms may
Congenital include delayed bleeding, ecchymosis, deep
Glanzmann thrombasthenia hematomas, epistaxis, spontaneous gingival
von Willebrand’s disease bleeding and hemarthrosis. A factor VIII
Acquired level of 6% to 50% of normal factor activity
Drug-induced (mild hemophilia) is associated with bleeding
Liver disease during surgery or trauma; 1% to 5% with
Alcoholism bleeding after mild injury; and < 1% (severe
Vascular disorders Scurvy hemophilia) with spontaneous bleeding. 3
Purpura Management of hemophilia A among pa-
Hereditary hemorrhagic telangiectasia tients undergoing dental surgery consists of 2
Cushing syndrome increasing factor VIII levels, replacing factor
Ehlers-Danlos syndrome VIII and inhibiting fibrinolysis (Table 2).
Fibrinolytic defects Streptokinase therapy Desmopressin (DDAVP) is used to achieve a
Disseminated intravascular coagulation transient increase in factor VIII level through
the release of endogenous factor VIII in pa-
tients with hemophilia A and von Willebrand’s
A general examination of the patient might in- disease. It may be sufficient to achieve hemostasis in mild
dicate a tendency to bleed. Multiple purpurae of forms of these diseases. DDAVP may be combined with an-
t he sk in, bleeding wounds, ev ident hematomas tifibrinolytic agents to increase its effectiveness. 2
or swollen joints may be evident in patients with Options for factor VIII replacement are factor VIII
severe bleeding defects. In addition, patients may show concentrates, fresh frozen plasma and cryoprecipitate.
signs of underlying systemic disease. Patients with liver di- Highly purified forms of factor VIII concentrates, manu-
factured using recombinant or monoclonal antibody puri-
sease may have jaundice, spider nevi, ascites and other signs
fication techniques, are preferred because of their greater
of impaired hepatic function. A cardiac patient can show
viral safety. 5,6 New generations of recombinant factor VIII
tachycardia or hypertension, which may make hemostasis
are being developed that are free from human and animal
more difficult to achieve. Evidence of petechiae, ecchy- proteins, in an attempt to further improve their safety.7 In
moses, hematomas or excessive gingival bleeding should di- patients who produce antibodies to factor VIII, a higher
rect the practitioner’s attention toward a possible underlying dose of concentrated factors can be considered, but a focus
bleeding disorder. When a bleeding disorder is suspected, on local measures is critical.
laboratory investigations, including blood counts and clot- Antifibrinolytic therapy can be used postoperatively
ting studies, should be carried out. Preoperative laboratory to protect the formed blood clot. Epsilon-aminocaproic
tests of the hemostatic system1,2 are: acid and tranexamic acid are the common agents used.

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Table 2 Presurgery treatment for hemophilia A4

Condition Treatment and dose Potential complications

Mild bleeding Dose: 15 U/kg factor VIII every 8–12 hours Hemarthrosis, oropharyngeal or dental bleeding,
for 1–2 days epistaxis, hematuria
Target: 30% of normal level

Major bleeding Dose: 50 U/kg factor VIII every 8–12 hours Same potential complications as for mild bleeding,
for 7–14 days as well as central nervous system hemorrhage,
Target: 80% to 100% of normal level retroperitoneal hemorrhage, gastrointestinal
bleeding
Adjunctive therapy Desmopressin, tranexamic acid or epsilon-
aminocaproic acid (for mild disease)

Table 3 Systemic diseases causing coagulopathies1

Disease Common causes Resulting coagulation defect

Renal failure and uremia Diabetes mellitus Inhibition of adhesion and primary aggregation
Glomerulonephritis of platelets from glycoprotein IIb–IIIa deficit
Pyelonephritis
Hypertension
Hepatic failure Alcohol abuse Obstructive jaundice: deficiency of vitamin
Hepatitis B and C K-dependent factors II, VII, IX and X
Cancer (e.g., hepatocellular Loss of liver tissue and all clotting factors except
carcinoma) VIII and von Willebrand’s factor
Bone marrow failure Alcohol abuse Reduced number of functioning platelets
Cancer (e.g., leukemia) Anemia from bone marrow suppression
Myelosuppressive medications
(e.g., chemotherapy for cancer)
Uremia from renal failure

Tranexamic acid in an oral rinse helps prevent postoperative tion.11 These patients may have a variety of bleeding dis-
bleeding from surgical wounds. Postoperative use of epsilon- orders depending on the extent of their liver disease.
aminocaproic acid can considerably reduce the level of factor Management options for hemostatic defects in liver disease5
required to control bleeding when used in conjunction with include vitamin K and fresh frozen plasma infusion (im-
presurgical infusion of factor VIII concentrate.8–10 mediate but temporary effect) for prolonged prothrombin
Hemophilia B is the result of factor IX deficiency. It is time and partial thromboplastin time; cryoprecipitate for
managed by replacement therapy with highly purified, virally replacement of factor VIII deficiency; and replacement the-
inactivated factor IX concentrates. Prothrombin complex rapy for disseminated intravascular coagulation. Patients
concentrates can also be used for factor IX replacement. suffering from viral hepatitis are a potential source of cross
von Willebrand’s disease is the most common here- infection, and necessary precautions should be taken during
ditary coagulation disorder with an incidence of 1 in procedures. Drug doses frequently need to be modified in
10,000. It is not sex linked. It is classified as Type I to these patients due to impaired liver function. The patient’s
Type IV and may vary in severity. For mild conditions, physician should be consulted before making any changes
use of DDAVP may be sufficient, but severe disease in the drug regimen.
warrants factor VIII replacement. Coagulopathies can be drug induced. Warfarin, low-
Other than congenital diseases, coagulation de- molecular-weight heparin and dicumarol (coumadin) are
fects may be acquired and from a variety of sources the most commonly used anticoagulant drugs. Treatment
(Table 3). In liver diseases, the synthesis of clotting factors must be modified in accordance with the medications that
may be reduced due to parenchymal damage or obstruc- the patient is taking and their impact on coagulation.

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Table 4 Principal agents for systemic management of patients with bleeding disorders3

Agent Description Common indications

Platelets 1 unit = 50 mL; may raise count by 6,000 Platelet count
< 10,000 in nonbleeding individuals
< 50,000 presurgical level
< 50,000 in actively bleeding individuals
Nondestructive thrombocytopenia
Fresh frozen plasma 1 unit = 150–250 mL Undiagnosed bleeding disorder with
1 hour to thaw active bleeding
Contains factors II, VII, IX, X, XI, XII, XIII Severe liver disease
and heat-labile V and VII When transfusing > 10 units of blood
Immune globulin deficiency
Cryoprecipitate 1 unit = 10–15 mL Hemophilia A, von Willebrand’s disease,
when factor concentrates and DDAVP
are unavailable
Fibrinogen deficiency
Factor VIII concentrate 1 unit raises factor VIII level 2% Hemophilia A with active bleeding or
Heat-treated contains von Willebrand’s presurgery; some cases of von Willebrand’s
factor disease
Recombinant and monoclonal technologies
are pure factor VIII
Factor IX concentrate 1 unit raises factor IX level 1–1.5% Hemophilia B, with active bleeding or
Contains factors II, VII, IX and X presurgery
Monoclonal formulation contains only Prothrombin complex concentrates used
factor IX for hemophilia A with inhibitor
Desmopressin Synthetic analogue of antidiuretic hormone Active bleeding or presurgery for some
0.3µg/kg IV or SC patients with von Willebrand’s disease,
Intranasal application uremic bleeding of liver disease,
bleeding esophageal varices
Epsilon-aminocaproic acid Antifibrinolytic: 25% oral solution Adjunct to support clot formation for any
(250 mg/mL) bleeding disorder
Systemic: 75 mg/kg every 6 hours
Tranexamic acid Antifibrinolytic: 4.8% mouth rinse (not Adjunct to support clot formation for any
available in the United States) bleeding disorder
Systemic: 25mg/kg every 8 hours

Note: IV = intravenous; SC = subcutaneous.

Platelet disorders can be hereditary or acquired and may cytopenic purpura, an acquired platelet disorder, oral sys-
be due to decreased platelet production, excess consumption temic steroids may be prescribed 7–10 days before surgery
or altered function. The most common clinical features are to increase the platelet count to safe levels.12 Patients with
bleeding from superficial lesions and cuts, spontaneous gin- Glanzmann thrombasthenia, an autosomal recessive di-
gival bleeding, petechiae, ecchymosis and epistaxis. sorder causing a defect in platelet aggregation, are given
The minimum blood platelet level before dental surgical
platelet infusion before surgery.
procedures is approximately 50,000/µL; extensive surgery
A number of drugs interfere with platelet function
may require > 100,000/µL. Replacement therapy may be
required if the count is below this level. Usually, platelet (see Appendix A at www.cda-adc.ca/jcda/vol-73/issue-
transfusion is carried out 30 minutes before surgery. In 1/77.html). Acetylsalicylic acid (ASA) and dipyridamole
patients with platelet levels below 100,000/µL prolonged are used therapeutically for platelet function inhibition.
oozing may occur, but local measures are usually sufficient Discontinuation of these drugs is not required for routine
to control the bleeding. In cases of idiopathic thrombo- procedures.

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Table 5 Local hemostatic agents minimize the challenge to the patient by restoring the
hemostatic system to acceptable levels and maintaining
Brand name Generic name or description
hemostasis by local and adjunctive methods. The patient’s
Gelfoam (Pfizer, Absorbant gelatin sponge material physician should be consulted before invasive treatment is
Markham, Ont.) undertaken. In patients with drug-induced coagulopathies,
Bleed-X (QAS, Microporous polysaccharide drugs may be stopped or the doses modified. For irreversible
Orlando, Fla.) hemispheres coagulopathies, replacement of missing factors may be ne-
Surgicel (Ethicon, Oxidized cellulose cessary (Table 4).
Markham, Ont.)
Pain Control
Tisseel (Baxter, Fibrin sealant
In patients with coagulopathies, nerve-block anesthetic
Mississauga, Ont.)
injections are contraindicated unless there is no better
Thrombostat (Pfizer) Topical thrombin alternative and prophylaxis is provided, as the anesthetic
Cyklokapron (Pfizer) Tranexamic acid solution is deposited in a highly vascularized area, which
Amicar (Wyeth, Epsilon-aminocaproic acid carries a risk of hematoma formation.14,15 The commonly
Markham, Ont.) 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 pro-
Vascular defects are rare and usually associated with duce gross swelling, pain, dysphasia, respiratory obstruction
mild bleeding confined to skin or mucosa.13 Scurvy, heredi- and risk of death from asphyxia.16–18 Anesthetic infiltration
tary hemorrhagic telangiectasia and other vascular defects and intraligamentary anesthesia are potential alternatives to
are usually treated with laser ablation, embolization or coa- nerve block in many cases. An anesthetic with a vasocons-
gulation. Recognizing vascular lesions during examination, trictor should be used when possible. Alternative techniques,
aspiration or advanced imaging may lead to modification of including sedation with diazepam or nitrous oxide–oxygen
treatment planning. analgesia, can be employed to reduce or eliminate the need
Fibrinolytic defects may occur in patients on medical for anesthesia. Patients undergoing extensive treatment
therapy and those with coagulation syndromes where fi- requiring factor replacement may be treated under general
brin is consumed (disseminated intravascular coagulation). anesthesia in a hospital operating room.
Recognition is important and oral care must be managed in
Oral Surgery
consultation with a hematologist.
Surgical procedures carry the highest risk of bleeding,
and safety precautions are needed. For coagulopathies,
Oral Findings
transfusion of appropriate factors to 50% to 100% of normal
Platelet deficiencies can cause petechiae or ecchymosis
levels is recommended when a single bolus infusion is used
in oral mucosa and promote spontaneous gingival bleeding. in an outpatient setting. In patients with hemophilia, ad-
These disorders may be present alone or in conjunction with ditional postoperative factor maintenance may be required
gingival hyperplasia in cases of leukemia. Hemosiderin and after extensive surgeries. This can be done with factor infu-
other blood degradation products can cause brown deposits sion, DDAVP, cryoprecipitate or fresh frozen plasma depen-
on the surface of teeth due to chronic bleeding. ding on the patient’s condition. The patient’s hematologist
People with hemophilia may have multiple bleeding should be consulted before planning, and patients with
events over their lifetime. The frequency of bleeding de- severe disease should be treated in specialty centres.
pends on the severity of hemophilia. Hemarthrosis of the Local hemostatic agents (Table 5) and techniques
temporomandibular joint is uncommon. 3 such as pressure, surgical packs, sutures and surgical
The incidence of dental caries and periodontal diseases stents may be used individually or in combination and may
is higher in patients with bleeding disorders, which may be assist in the local delivery of hemostatic agents, such as
because of lack of effective oral hygiene and professional topical thrombin and vasoconstrictors. However, caution is
dental care due to fear of oral bleeding. needed with the use of vasoconstrictors because of the risk
of rebound vasodilatation, which may increase late bleeding
Dental Management risk. The use of absorbable hemostatic materials may favour
The management of patients with bleeding disorders clot formation and stability. However, these materials also
depends on the severity of the condition and the invasive- carry a risk of infection and may delay healing; they should
ness of the planned dental procedure. If the procedure has therefore be avoided in immunosuppressed patients. Topical
limited invasiveness and the patient has a mild bleeding thrombin is an effective agent when applied directly on
disorder, only slight or no modification will be required. the bleeding wound as it converts fibrinogen to fibrin and
In patients with severe bleeding disorders, the goal is to allows rapid hemostasis in a wound. Topical fibrin glue can

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reduce the amount of factor replacement needed when used Restorative and Endodontic Procedures
along with antifibrinolytic agents.19–22 Fibrin glue has also General restorative procedures do not pose a significant
been effectively used in conjunction with other hemostatic risk of bleeding. Care should be taken to avoid injuring the
measures. gingiva while placing rubber dam clamps, matrices and
The use of drugs affecting bleeding mechanisms does wedges. A rubber dam should be used to prevent laceration
not usually pose a significant problem in dental treatment. of soft tissues by the cutting instruments. Saliva ejectors
If ASA has to be withdrawn, this should be done at least 10 and high-speed suction can injure the mucosa in the floor
days before surgery. In most cases, ASA therapy does not of the mouth and cause hematoma or ecchymosis; thus, they
need to be stopped, and local hemostatic measures are suffi- should be used carefully.
cient to control bleeding. Similarly, other antiplatelet drugs, Endodontic therapy is preferred over extraction whe-
such as clopidogrel and dipyridamole, usually do not need never possible in these patients. Endodontic therapy does
to be stopped. The patient’s physician should be consulted not usually pose any significant risk of bleeding and can be
before any decision is made to modify the patient’s drug performed routinely. Endodontic surgical procedures may
regimen, and the potential risk–benefit ratio should be de- require factor replacement therapy.
termined. For patients taking warfarin, their international
Prosthodontic Procedures
normalized ratio (INR) should be measured before a sur-
These procedures do not usually involve a considerable
gical procedure. The normal therapeutic range is 2.0–3.0.
risk of bleeding. Trauma should be minimized by careful
According to current recommendations, most oral surgical
post-insertion adjustments. Oral tissue should be handled
procedures can be performed without altering the warfarin
delicately during the various clinical stages of prosthesis
dose if the INR is less than 3.0. 23 If INR values are greater
fabrication to reduce the risk of ecchymosis. Careful ad-
than 3.0, physician referral is suggested. It is important to
justment of prostheses is needed to reduce trauma to soft
consider the risk of reducing the level of anticoagulation in tissue.
patients on warfarin due to the risk of a thromboembolic
event.24 Patients taking heparin are often those who are on Orthodontic Procedures
hemodialysis due to end-stage renal disease. Heparin has Orthodontic therapy can be carried out without blee-
a short half-life (about 5 hours) and patients can often be ding complications, although care should be taken that ap-
treated safely on the days between dialysis. pliances do not impinge on soft tissues and emphasis should
be put on excellent, atraumatic oral hygiene.
Periodontal Procedures
Periodontal health is of critical importance in patients Choice of Medications
with bleeding disorders3 as inflamed and hyperemic gin- Many medications prescribed in dental practice,
gival tissues are at increased risk of bleeding. Periodontitis especially ASA, may interfere with hemostasis. In
may cause tooth mobility and warrant extraction, which addition, many drugs interact with anticoagulants, in-
may be a complicated procedure in these patients. Patients creasing their potency and the risk of bleeding. When
with coagulopathies may neglect their oral health due to used for prolonged periods, ASA and nonsteroidal anti-
fear of bleeding during tooth brushing and flossing, which inflammatory drugs (NSAIDS) can increase the effect of
leads to increased gingivitis, periodontitis and caries. warfarin. Penicillins, erythromycin, metronidazole, te-
Periodontal probing, supragingival scaling and polishing tracyclines and miconazole also have potentiating
can be done normally without the risk of significant blee- effects on warfarin. Care should be taken when prescribing
ding. Factor replacement is seldom needed for subgingival these drugs to patients with bleeding tendencies or those
receiving anticoagulant therapy, and it may be desirable
scaling and root planing if these procedures are done care-
to consult the patient’s physician before planning the dose
fully. Ultrasonic instrumentation may result in less tissue
regimen. a
trauma. For severely inflamed tissues, initial treatment with
chlorhexidine mouthwashes and gross debridement is re-
commended to reduce tissue inflammation before deep sca- THE AUTHORS
ling.25 Factor replacement may be required before extensive
periodontal surgery and use of nerve blocks. Periodontal
Dr. Gupta is a dental student at Tufts University in Boston,
packing materials and custom vinyl mouthguards (stents) Massachussetts.
are used to aid in hemostasis and protect the surgical site,
but these can be dislodged by severe hemorrhage or subpe-
riosteal hematoma formation. 3 Antifibrinolytic agents may
be incorporated into periodontal dressings for enhanced Dr. Epstein is professor and head, department of oral medicine
and diagnostic sciences, Chicago Cancer Center, University of
effect. Post-treatment antifibrinolytic mouthwashes are Illinois, Chicago, Illinois.
usually effective in controlling protracted bleeding.

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 ––– Patients with Bleeding Disorders –––

Dr. Cabay is a resident physician, department of pathology,

College of Medicine, University of Illinois at Chicago, Chicago,
Illinois.

Correspondence to: Dr. Joel B. Epstein, Department of Oral Medicine and

Diagnostic Sciences, College of Dentistry, University of Illinois at Chicago,
801 S. Paulina St., M/C 838, Chicago, IL 60612-7213, USA.

The authors have no declared financial interests in any company manufac-

turing the types of products mentioned in this article.

This article has been peer reviewed.

References
The complete list of references is available in the electronic version of this
article at www.cda-adc.ca/jcda/vol-73/issue-1/77.html.

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