Hemophilia A and B

Overview

Board takeaway: Agree on hemostasis with hematology before every invasive step: blocks, extractions, and scaling. Hemophilia comprises X-linked factor deficiencies.

How do Hemophilia A and B affect dental management?

• Prevention first: aggressive hygiene, fluoride and sealants reduce the need for invasive care
• Local anesthesia: buccal infiltration and intraligamentary injections are lower-risk alternatives. Inferior alveolar, superior alveolar, and floor-of-mouth or vascular lingual injections require preoperative systemic hemostatic measures agreed with hematology to prevent hematoma. WFH recommendations 2.7.10–11.
• Isolation: rubber dam with careful clamp placement.
• Tooth prognosis: preserve a restorable tooth when the pulpal diagnosis and prognosis support it. Do not retain an infected, nonrestorable tooth solely to avoid extraction.
• Extraction: hematology-directed hemostatic support, gentle technique, local hemostatics (gelatin sponge, oxidized cellulose, sutures), and tranexamic acid rinse or tablets. WFH recommendations 2.7.7–9.
• Analgesia: acetaminophen is the usual first choice; avoid aspirin and nonselective NSAIDs.
• Alternatives: any alternative, including a selective COX-2 inhibitor or rarely an opioid, requires an individualized medical plan.

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Invasive care requires a personalized hematology plan. Deep blocks and vascular lingual or floor-of-mouth injections require preoperative systemic hemostatic measures; lower-risk infiltration or intraligamentary routes still need individualized assessment.

Exam pearl

What are Hemophilia A and B?

Inherited bleeding disorders, usually X-linked, caused by deficient clotting factor VIII (hemophilia A) or factor IX (hemophilia B), resulting in impaired clot formation and prolonged bleeding.

What are the main systemic features of Hemophilia A and B?

• Inheritance: X-linked inheritance; predominantly affects males, but females with low factor levels can have clinically important bleeding and require the same individualized assessment
• Hemophilia A: factor VIII deficiency; ~85% of cases; about 1 in 5,000 male births
• Hemophilia B: factor IX deficiency; about 1 in 30,000 male births
• Severity by factor level: severe under 1%, moderate 1–5%, mild 5–40%
• Typical labs: prolonged activated partial thromboplastin time (aPTT), with normal prothrombin time (PT) and platelets.
• Bleeding pattern: hemarthrosis and deep muscle bleeding.
• Procedural assessment: the bleeding history and factor diagnosis remain essential; a routine clotting test alone is not clearance.
• Inhibitors: antibodies to factor complicate replacement.
• Prophylaxis: emicizumab is used in hemophilia A.
• DDAVP: may raise factor VIII sufficiently in responsive mild, and selected moderate, hemophilia A; confirm response with hematology. It does not treat factor IX deficiency.

What oral findings occur in Hemophilia A and B?

• Prolonged bleeding after exfoliation, extraction or frenum laceration (labial frenum the most common site)
• Gingival bleeding and poor hygiene from fear of brushing; caries risk
• Ecchymoses and hematomas; prominent petechiae or mucosal bleeding should also prompt consideration of a platelet disorder or von Willebrand disease

For Hemophilia A and B, when is DDAVP an alternative to factor replacement?

Only when the hematology team has established an adequate response for the planned procedure. Desmopressin releases stored von Willebrand factor and factor VIII; it may be used in responsive mild or selected moderate hemophilia A and responsive type 1 von Willebrand disease.

• Hemophilia B: ineffective because it does not raise factor IX.
• Safety: contraindicated under age two; follow prescribed fluid restriction and sodium precautions because water retention can cause hyponatremia and seizures.
• Coverage: the procedure, required factor rise, duration, and response determine whether factor replacement is still needed.

A 6-year-old with severe hemophilia A needs two primary molar extractions. What is your treatment plan?

1. Confirm the hematology plan: severity, inhibitor status, current prophylaxis, and written coverage for both extractions and local anesthesia.
2. Confirm systemic support: hematology specifies any factor or bypassing-agent support, timing, and duration. Emicizumab prophylaxis alone does not establish procedural coverage and is not treatment for an acute bleed.
3. Control local bleeding: atraumatic technique; local hemostatics such as gelatin sponge, oxidized cellulose, and sutures; tranexamic acid rinse or oral treatment for several days.
4. Plan aftercare: acetaminophen, a soft diet, clear clot-protection instructions, and contact/reassessment arrangements for delayed bleeding.
5. Escalate: persistent bleeding despite pressure or expanding oral/neck swelling requires urgent specialist or emergency care.

For Hemophilia A and B, which local anesthetic injections can you give without factor coverage?

• Lower-risk alternatives: buccal infiltration and intraligamentary injections. Confirm the individual hemostasis plan with hematology rather than assuming every injection can be given without coverage.
• Higher-risk injections: inferior alveolar and superior alveolar blocks, and injections into the floor of the mouth or vascular lingual tissues, need preoperative systemic hemostatic measures planned with hematology. A deep hematoma can compromise the airway.

For Hemophilia A and B, what is an inhibitor and why does it matter?

• What it is: an antibody against the infused factor, more common in severe hemophilia A.
• Replacement: ordinary factor replacement may be ineffective; the hemophilia team determines factor/bypassing-agent treatment and the setting.
• Emicizumab: prevents bleeding in hemophilia A but does not treat an acute bleed. Concomitant activated prothrombin complex concentrate can cause serious thrombotic complications.
• Setting: a complex invasive plan may require hospital resources.

Sources

• Supplemental hematology guidance: WFH Guidelines for the Management of Hemophilia, 3rd edition: Chapter 2
• Supplemental hematology guidance: WFH Guidelines, 3rd edition: desmopressin
• Supplemental systemic features: MedlinePlus Genetics: hemophilia
• Supplemental study guide: Pediatric Dental Study Guide: Hemophilia
• Common Laboratory Values
• Hematology Consultation for Pediatric Dental Patients