Inherited Bleeding Disorders

Question 1

What is haemophilia A?

Answer 1

Deficiency of factor 8 clotting factor.
Sex-linked recessive.

Question 2

What is haemophilia B?

Answer 2

Deficiency of factor 9 clotting factor.
Sex-linked recessive.

Question 3

What is Von Willebrand’s disease?

Answer 3

Deficiency of factor 8 clotting factor plus reduced platelet aggregation caused by a deficiency of von willebrand factor.
Autosomal dominant.

Question 4

What factor levels make haemophilia severe, moderate mild or carriers?

Answer 4

Severe- less than 0.02iu/ml
Moderate- 0.02-0.09iu/ml
Mild- 0.1-0.4iu/ml
Carriers- more than 0.05iu/ml

Question 5

What medication might someone with Haemophilia A be on?

Answer 5

Severe or moderate- recombinant factor 8.
Mild or carriers- DDAVP or tranexamic acid.

Question 6

What is DDAVP and tranexamic acid?

Answer 6

DDAVP- removes coagulation factor 8 from arterial wall.
Tranexamic acid- inhibits fibrinolysis.

Question 7

What medication might someone be on if they have Haemophilia B?

Answer 7

Recombinant factor 9 regardless of status.

Question 8

What are coagulation factor inhibitors?

Answer 8

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.

Question 9

What LA techniques are safe for haemophilia patients?

Answer 9

Buccal infiltrations
Intra-papillary
Intra-ligamental

Question 10

Which LA techniques are not safe for haemophilia patients?

Answer 10

IDB
Lingual infiltration
Posterior superior nerve block

Question 11

What type of procedures are allowed in general practice for haemophilia patients?

Answer 11

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.

Question 12

What procedures would require to be done in a haemophilia centre?

Answer 12

Anything involving the gingival margin.
Extractions, PMPR, sub gingival restorations, crowns, bridges requiring a block, flap-raising procedures, biopsies.
LA- IDBs

Question 13

What value of platelets is required for the patient to be treated in general practice?

Answer 13

over 100x10 to the 9/L.

Question 14

What value of platelets is required for a patient to be treated in hospital?

Answer 14

50-100x10 to the 9/L

Question 15

What procedures are fine if the platelet count is grater than 100?

Answer 15

Hygiene therapy
Prosthodontics
Crowns, bridges, restorations
Endodontics
Ortho treatment

If surgery is considered, then seek advice for the haematologist.

Question 16

What is the patient likely to require to take prior to invasive treatment if they are mild sufferer of haemophillia or VWD?

Answer 16

Desmopressin.

Question 17

What is the patient likely to require to take prior to invasive treatment if they are a moderate or severe haemophilia sufferer?

Answer 17

Recombinant factor.

Question 18

Why is is desirable to reduce the number of times a patient has to take recombinant factor?

Answer 18

Risk of local site infection.
Risk of inhibitors developing.
Cost.

Question 19

What tests may you wish to request prior to undertaking any treatment in these patients?

Answer 19

Full blood count.

Question 20

What is Haemarthrosis?

Answer 20

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.

Question 21

What type of inheritance is Haemophillia?

Answer 21

X-linked recessive

Question 22

What type of inheritance is VWD?

Answer 22

Autosomal dominant

Question 23

Why do you want to avoid giving the patient factor replacement too often?

Answer 23

Risk of blood borne infections
Risk of inhibitors/antibodies developing
Risk of local site infection
Cost