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Diseases of the Blood BDS2 > Inherited Bleeding Disorders > Flashcards

Inherited Bleeding Disorders Flashcards

1
Q

what is an inherited bleeding disorders?

A

an acquired defect which affects the coagulation of the blood

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2
Q

what may an inherited bleeding disorder affect?

A

coagulation cascade, platelets, a combined deficiency

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3
Q

what is affected with coagulation cascade disorders?

A

reduction in one or more of the coagulation factors or control proteins

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4
Q

what is affected with platelet disorders?

A

number or function

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5
Q

what is factor VIII deficiency called?

A

haemophilia A

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6
Q

what is factor IX deficiency called?

A

christmas disease/haemophilia B

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7
Q

what is von willebrand’s disease?

A

reduced factor VIII level and reduced platelet aggregation

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8
Q

how does haemophilia A and B come about?

A

inheritance with a sex-linked recessive gene - defective gene on X chromosome

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9
Q

what is the severity of haemophilia due to?

A

the amount of factor produced where 1iu is normal

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10
Q

in relation to iu what is severe haemophilia?

A

less than 2% = <0.02iu/ml

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11
Q

in relation to iu what is moderate haemophilia?

A

2-9% = 0.02-0.09iu/ml

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12
Q

in relation to iu what is mild haemophilia?

A

10-40% = 0.1-0.4iu/ml

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13
Q

what is the iu of carriers of haemophilia?

A

> 50% = >0.5iu/ml

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14
Q

what is the treatment of severe and moderate haemophilia A?

A

require the use of recombinant factor VIII

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15
Q

what is the treatment of mild and carrier haemophilia A?

A

DDAVP and tranexamic acid

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16
Q

what does DDAVP do?

A

release factor VIII that has been bound to endothelial cells giving a temporary boost to factor VIII levels and clotting ability

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17
Q

what does tranexamic acid do?

A

inhibitor of fibrinolysis - keeps any clot that is formed

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18
Q

what is the treatment of severe and moderate haemophilia B?

A

recombinant factor IX

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19
Q

what is the treatment of mild and carriers of haemophilia B

A

recombinant factor IX

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20
Q

why does all severities of haemophilia B require recombinant factor IX

A

because factor IX does not bind to the endothelial cell wall so DDAVP will not work

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21
Q

what are coagulation factor inhibitors?

A

antibodies which develop to factor VIII and IX

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22
Q

what is von willebrands disease caused by?

A

autosomal dominant (not X chromosome related)

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23
Q

with von willebrands disease why is there poor clot activation by platelets?

A

because there is defective von Willebrand factor on platelets which interacts badly with factor VIII

24
Q

what is type 1 von willebrand disease?

A

dominant mild

25
what is type 2 von willebrand disease?
dominant mild
26
what is type 3 von willebrand disease?
recessive severe
27
what is treatment for severe and moderate von willebrands disease?
DDAVP
28
what is treatment for mild and carriers of von willebrands disease?
oral tranexamic acid
29
what is the most common bleeding disorder in the UK?
von willebrands disease
30
what are rarer bleeding disorders?
inherited defects of other factors in the coagulation pathway or of either the number or function of the platelets
31
what procedures can be done in primary care for bleeding disorder patients?
non-bleeding procedures
32
what does the degree of bleeding risk depend on?
baseline factor activity - higher factor number gives lower risk
33
what must the patient treatment plan incorporate?
both patient and treatment risks
34
where are the scottish haemophilia centres?
inverness, aberdeen, dundee, edinburgh, glasgow
35
where does dental care for severe and moderate haemophiliacs take place?
in the dental treatment unit attached to haemophilia centre (apart from pros)
36
where does dental care for mild and carriers of haemophilia take place?
treatment shared by GDP/PDS depending upon procedure and LA needs
37
what dental procedures require special care?
administration of LA, extractions, minor oral surgery, periodontal surgery, biopsies
38
what types of LA are safe in haemophilia?
buccal infiltration, intraligamentary, intra-papillary injections
39
what types of LA are dangerous in haemophilia?
IDB, lingual infiltration, posterior superior nerve block
40
what is the most important aspect of care for patients with medical issues?
prevention - dietary advice, oral hygiene, fluoride supplements, regular dental care, fissure sealant where appropriate
41
what is thrombophilia?
increased risk of clots developing, clot formation greater than clot breakdown
42
what happens if a blood clot embolises?
lead to blockage of major blood vessels in the heart and/or lungs - pulmonary thromboembolism
43
what are the inherited hypercoagulation syndomes?
protein C deficiency, protein S deficiency, factor V lieden, antithrombin III deficiency
44
what are the acquired hypercoagulation risks?
antiphospholipid syndrome, oral contraceptives, surgery, trauma, cancer, pregnancy, immobilisation
45
what is thrombocytopenia?
reduced platelet numbers
46
what is qualitative disorders?
normal platelet number but abnormal function
47
what is thrombocythemia?
increased platelet numbers
48
what is the cause of thrombocytopenia caused by?
idiopathic, drug related (alcohol, penicillin, heparin), secondary to lymphoproliferative disorder (leukaemia, myelodysplasia)
49
what does platelet count need to be greater than in a primary care setting for treatment to proceed?
100 x 10^9/L
50
what does platelet count need to be greater than in a hospital setting for treatment to proceed?
50 x 10^9/L
51
what are thrombocythemia patients usually on?
aspirin to prevent clot formation
52
what inherited issues cause qualitative platelet disorders?
Bernard Soulier syndrome, Hermansky Pudlak, Glanzmann's thrombasthenia
53
what acquired issues cause qualitative platelet disorders?
cirrhosis, drugs, alcohol, cardiopulmonary bypass
54
what procedures can go ahead for platelet disorder patients with greater 100 x10^9/L platelets?
hygiene therapy, removable prosthodontics, restorative dentistry, endodontics, orthodontic treatment
55
if platelet count is below 100 x 10^9/L or above 500 x10^9/L what procedures require special care?
extractions, minor oral surgery, periodontal surgery, biopsies

Diseases of the Blood BDS2 (8 decks)

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