Inherited bleeding disorders Flashcards
How should you as a dentist, treat a patient with an inherited bleeding disorder?
- Treat as if the are a normal person (on the most part)
- May need treatment planning
- Be aware of potential problems: (hygiene phase therapy, LA, extractions and surgery)
How do you prevent someone with an inherited bleeding disorder from needing dental treatment?
PREVENTION:
- OH
- Regular dental care
- Fluoride supplements
- Fissure sealant
- Dietary advice
Which dental treatments are there no additional problems with, with someone with an inherited bleeding disorder? (5 points)
- Hygiene therapy
- Removable prosthodontics
- Restorative dentistry, including crowns and bridges
- endodontics
- Orthodontic treatment
(anything that does not involve blood coming from the mouth)
What dental treatments require special care for people with inherited bleeding disorders? (4 points)
- Extractions
- Minor oral surgery
- Periodontal surgery
- Biopsies
What precautions should you put in place for people with inherited bleeding disorders that are getting an extraction or surgery? (5 points)
- Appropriate monitoring +/- treatment prior to the procedure
- Atraumatic treatment
- Consider antibiotics
- Observe for to ensure haemostasis
- Comprehensive post-operative instructions
What is an inherited bleeding disorder?
An acquired defect which affects the coagulation of the blood
What processes may inherited bleeding disorders affect? (3 points)
- Coagulation cascade (a reduction in one, or more, of the coagulation factors)
- Platelets (number, function)
- A combined deficiency (in some cases we have both causing a problem)
What are the most common inherited blood disorders? (4 points)
- Factor VIII deficiency
- Factor IX deficiency
- Von Willebrand’s disease
- Factor XI deficiency
Which disorders are caused by a factor VIII deficiency?
- Haemophilia
- Haemophilia A
Which disorders are caused by a factor IX deficiency?
Haemophilia B
What is Von Willebrand’s disease? (2 points)
No other name
- Reduced factor VIII level
- Reduced platelet aggregation
Which population is factor XI deficiency common in?
Common in Ashkenazy Jew population
What are rare bleeding disorders caused by? (3 points)
- Inherited defects of other factors in the coagulation pathway
- Inherited defect of either the number or function of the platelets
- Numbers in each group are small
Why is management of rare bleeding disorders complex?
Management in complex due to the bleeding does not always relate to the factor levels
What kind of inheritance causes a rare coagulation disorder?
- Autosomal recessive inheritance
- Generally, lack of clear correlation between bleeding and level of factor, so more difficult to manage
What are inhibitors?
- These are antibodies which develop to factor VIII and IX
- The amount of antibody developed varies between patients
What % of patients develop an inhibitor when they first start treatment (artificial clotting factors)?
- 30%-40%
- Usually disappear shortly after treatment
What type of inheritance is haemophilia A & B?
- Sex-linked recessive
- Males are affected and females are carriers
What would your levels of clotting factors be in units for someone with severe haemophilia?
<0.02 iu/ml
What would your levels of clotting factors be in units for someone with moderate haemophilia?
0.02-0.09 iu/ml
What would your levels of clotting factors be in units for someone with mild haemophilia?
0.1-0.4 iu/ml
What would your levels of clotting factors be in units for someone who is a carrier for haemophilia?
<0.5 iu/ml
What would your levels of clotting factors be in units for someone who has normal levels?
1
What treatment would be given to people with severe/moderate haemophilia A?
Require the use of recombinant factor VIII
What treatment would be given to people with mild/carrier haemophilia A?
- The majority of patients will respond to DDAVP
- Very mild cases may only require oral tranexamic acid
- DDVPA is a vasopressin works like ADH (in the blood stream F8 tends to stick to endothelial cells so there is always F8 in the cells which are bound and cannot do anything. DDAVP will release them to allow them to work)
- Tranexamic acid - slows down the rate at which the clot you make is lost can re return to balance
Do people with haemophilia B respond to DDAVP?
no - not going to help as they don’t have a problem with F8
- Have a F9 deficiency so the only way to treat this is with prophylactic cover
What is the treatment option for people with haemophilia B?
Prophylactic cover requires recombinant factor IX
What type of inheritance is Von Willebrand’s disease?
- Autosomal dominant
- Both sexes are equally affected
What is Von Willebrand’s disease?
Deficiency of Von Willebrand factor with a reduction in factor VIII levels
Is type 1 VWD dominant or recessive?
- dominant
- Produces a mild form of the disease
Is type 2 VWD dominant or recessive?
- Dominant
- Produces a mild form of the gene
Is type 3 VWD dominant or recessive?
- Recessive
- Produces a severe form of the gene
Do patients with VWD respond to DDAVP?
- Yes
- Very mild cases may only require oral tranexamic acid
Where do people with severe & moderate inherited bleeding disorders get dental treatment?
Majority of the treatment in the hospital except for prosthodontics
- Treatment by GDP/PDS by arrangement
Where do people with carriers and mild inherited bleeding disorders get dental treatment?
- Treatment shared with GDP/PDS
- Patient reviewed at the hospital every 2 years
When is special care dental treatment required for people with inherited bleeding disorders? (5 points)
- Administration of LA
- Extractions
- Minor oral surgery
- Periodontal surgery
- Biopsies
Which LA injection sites are safe for a patient with an inherited bleeding disorder? (3 points)
- Buccal infiltration
- Intraligamentary injections
- Intra-papillary injections
Which LA injection sited are dangerous for a patient with an inherited bleeding disorder? (3 points)
- Inferior alveolar nerve block
- Lingual infiltration
- Posterior superior nerve block
What precautions should be taken for a patient with an inherited bleeding disorder when they need an extraction or surgery? (5 points)
- Appropriate cover from haemophilia unit
- Atraumatic treatment
- Consider antibiotics
- Observe for 2-3 hours after surgery (minimum requirement)
- Comprehensive post-operative instructions
How long should severe and moderate patients be observed after extractions and surgery?
Patients should be observed overnight following surgery
How long should carrier and mild patients be observed after extractions and surgery?
Patients must be observed for 2-3 hours after surgery
Where should dentate patients with severe and moderate disease be treated?
Treatment in hospital or by arrangement with Haemophilia unit
Where should edentulous patients with severe and moderate disease be treated?
Treatment may be carried out in GDP or CDS
What is thrombophilia?
- Increased risk of clots developing
- Often an acquired condition superimposed on a genetic condition
What are inherited syndromes of thrombophilia? (4 points)
- Protein C deficiency
- Protein S deficiency
- Factor V Leiden
- Antithrombin III deficiency
What are examples of acquired syndromes of thrombophilia? (7 points)
- Antiphospholipid syndrome
- Oral contraceptives
- Surgery
- Trauma
- Cancer
- Pregnancy
- Immobilisation
What is thrombocytopenia?
Reduced platelet numbers
What are qualitative disorders?
Normal platelet number but abnormal function
What is thrombocythemia?
increased platelet number
What are the possible causes of thrombocytopenia?
- Idiopathic
- Drug related (alcohol, penicillin’s, heparin)
- Secondary to lymphoproliferative disorder
Dental treatment can proceed safely of patients with thrombocytopenia providing that the platelet count is what?
Provided the platelet count is >50x10^9
What are examples of rare inherited qualitative disorders? (3 points)
- Bernard Soulier Syndrome
- Hermansky Pudlak
- Glanzmann’s thrombasthenia
What are possible ways of getting acquired qualitative disorders? ( 4 points)
- Cirrhosis
- Drugs
- Alcohol
- Cardiopulmonary bypass
IS thrombocythemia a common or uncommon disease?
Uncommon
What treatment would you put someone with thrombocythemia on?
Usually on aspirin to prevent clot formation
