Inherited Bleeding Disorders Flashcards
What is haemophilia A?
Deficiency of factor 8 clotting factor.
Sex-linked recessive.
What is haemophilia B?
Deficiency of factor 9 clotting factor.
Sex-linked recessive.
What is Von Willebrand’s disease?
Deficiency of factor 8 clotting factor plus reduced platelet aggregation caused by a deficiency of von willebrand factor.
Autosomal dominant.
What factor levels make haemophilia severe, moderate mild or carriers?
Severe- less than 0.02iu/ml
Moderate- 0.02-0.09iu/ml
Mild- 0.1-0.4iu/ml
Carriers- more than 0.05iu/ml
What medication might someone with Haemophilia A be on?
Severe or moderate- recombinant factor 8.
Mild or carriers- DDAVP or tranexamic acid.
What is DDAVP and tranexamic acid?
DDAVP- removes coagulation factor 8 from arterial wall.
Tranexamic acid- inhibits fibrinolysis.
What medication might someone be on if they have Haemophilia B?
Recombinant factor 9 regardless of status.
What are coagulation factor inhibitors?
Antibodies which develop to factor 8 and factor 9.
Important to not give recombinant factor too often, otherwise the antibodies will increase.
Must give a higher dose each time to overcome the antibodies.
What LA techniques are safe for haemophilia patients?
Buccal infiltrations
Intra-papillary
Intra-ligamental
Which LA techniques are not safe for haemophilia patients?
IDB
Lingual infiltration
Posterior superior nerve block
What type of procedures are allowed in general practice for haemophilia patients?
Prosthodontics- if mild/severe and only if edentulous for moderate/severe patients.
Preventative care- diet advice, oral hygiene, fluoride supplements, fissure sealants, fluoride varnish application.
Supragingival restorations.
Crowns and bridges only requiring infiltrations.
Check ups.
What procedures would require to be done in a haemophilia centre?
Anything involving the gingival margin.
Extractions, PMPR, sub gingival restorations, crowns, bridges requiring a block, flap-raising procedures, biopsies.
LA- IDBs
What value of platelets is required for the patient to be treated in general practice?
over 100x10 to the 9/L.
What value of platelets is required for a patient to be treated in hospital?
50-100x10 to the 9/L
What procedures are fine if the platelet count is grater than 100?
Hygiene therapy
Prosthodontics
Crowns, bridges, restorations
Endodontics
Ortho treatment
If surgery is considered, then seek advice for the haematologist.
What is the patient likely to require to take prior to invasive treatment if they are mild sufferer of haemophillia or VWD?
Desmopressin.
What is the patient likely to require to take prior to invasive treatment if they are a moderate or severe haemophilia sufferer?
Recombinant factor.
Why is is desirable to reduce the number of times a patient has to take recombinant factor?
Risk of local site infection.
Risk of inhibitors developing.
Cost.
What tests may you wish to request prior to undertaking any treatment in these patients?
Full blood count.
What is Haemarthrosis?
Bleeding into a joint space.
it can cause damage to the articular cartilage within the joint if it happens repeatedly.
May lead to bone resorption and cyst formation.
What type of inheritance is Haemophillia?
X-linked recessive
What type of inheritance is VWD?
Autosomal dominant
Why do you want to avoid giving the patient factor replacement too often?
Risk of blood borne infections
Risk of inhibitors/antibodies developing
Risk of local site infection
Cost
