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BDS2 BAMS > Inherited Bleeding Disorders > Flashcards

Inherited Bleeding Disorders Flashcards

1
Q

what is the timescale of the coagulation cascade

A

Continuous process happens all the time in everyone

Fibrin lysis is happening at same rate of coagulation

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2
Q

what is the relationship between clot build up and break down in a coagulopathy pt

A

Clotting and clot breakdown are not matched

- Imbalance in ability to clot or not clot

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3
Q

what is the role of proteins in the coagulation cascade

A

Areas of problems if deficient
Breaks on coagulation system
- Missing not behave the way they should
- Defined control is upset

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4
Q

how should you treat a bleeding disorder pt in general

A

Treat as a normal person
- Depend on individual and disease level

Treatment planning

Be aware of potential problems

  • Hygiene phase therapy
  • Local anaesthetic
  • Extractions and surgery
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5
Q

what is the main technique to employ for dental treatment of blood disorder pt

A

PREVENTION

Avoid having to do dentistry - then not a risk as problem cannot arise (manage with prevention)

  • Oral Hygiene
  • Regular dental care
  • Fluoride supplements
  • Fissure sealant
  • Dietary advice
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6
Q

what are dental treatments that special care would be required for treating a bleeding disorder pt (4)

A

Extraction

Minor oral surgery

Periodontal surgery

Soft tissue Biopsies

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7
Q

what are dental treatments that no additional problems would be incurred for a bleeding disorder pt

A

Hygiene therapy

Removable prosthodontics

Restorative dentistry inc crowns and bridges

Endodontics

Orthodontic treatment

No blood being released from mouth or blood getting into mouth
No need for ID block

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8
Q

what do you and the pt haematologist need to do prior to dental extractions and surgery

A

Appropriate monitoring +/- treatment prior to the procedure

Preparation work is necessary to get pt properly ready so more likely to have successful treatment

Liaise with haematologist so they work out coagulation issue for the dental tx plan
- Communicate well

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9
Q

what are the 3 possible areas of defect for the inherited bleeding disorders

A

coagulation cascade

platelets

a combined deficiency

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10
Q

what could be a coagulation cascade issue

A

a reduction in one, or more, of the coagulation factors

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11
Q

what could be issues with the platelets

A

number or function

- both are key to platelet role

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12
Q

Factor VIII deficiency

A

haemophilia

haemophilia A

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13
Q

factor IX deficiency

A

Christmas disease

haemophilia B

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14
Q

Von Willebrand’s disease

A

Reduced factor VIII level

Reduced platelet aggregation

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15
Q

factor XI deficiency effects

A

common in Ashkenazy Jew population

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16
Q

what are examples of types of rare bleeding disorders

A

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
- Large number of different conditions registered in the UK (Over 50)

Management is complex as the bleeding does not always relate to the factor levels

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17
Q

what is the representation of bleeding disorders like in small gene pools

A

disproportionately represented

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18
Q

what are carriers of Haemophilia

A

More than enough
- Large amount of redundancy in clotting factors in normal life

Serious bleeding problem where coagulation severely tested then can be an issue

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19
Q

what type of inheritance is HA and HB

A

sex linked recessive gene
- One X chromosome that is defective

Rare for girls
Boy with will have

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20
Q

what is the CF that is effected in haemophilia

A

CF8

Make some but inadequate if slightly broken
Completely defective – no F8 made

spectrum of activity depending on how defective the gene is

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21
Q

what are haemophilia centres

A

Manages all coagulopathies
Few in Scotland

Practically - rural have distance to travel to get to major support

General plan of action with GP and local hospital and then large problems at centre

Have a special dental care team attached

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22
Q

who are more prone to get rare coagulation disorders

A 
Autosomal recessive (?) inheritance
- Bad luck – 2 recessive genes

More common in racial groups where cousin marriage frequent
- Factor XI common in Ashkenazy Jews

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23
Q

why are rare coagulation disorders difficult to manage

A

Generally, lack of clear correlation between bleeding and level of factor, so more difficult to manage
- Doesn’t always translate into bleeding problems

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24
Q

what are inhibitors to CFs

A

antibodies which develop to factor VIII and IX (clotting factor)

  • Body doesn’t make them usually
  • Binds to artificial clotting factor (not made in body) so removed from circulation so no clotting factor
  • — Slightly different to body’s – matches the receptor and triggers the sequences but tail end can be subtly different so recognised as foreign

The amount of antibody developed varies between patients

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25
Q

when are inhibitors made

A

35-40% of patients develop an inhibitor when they first start treatment

Usually disappear shortly after treatment
- Use factor fast - fine as take a while for body to make response

But if give artificial clotting factor often then need to factor in part removed by inhibitor
- Give more to overcome the loss

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26
Q

how should pt be treated with artificial recombinant CFs to manage inhibitors

A

Need to have carefully Tx to allow inhibitors to drop most of the time so when given factors have a positive effect

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27
Q

what does a pt with a congenital bleeding disorder look like

A

Normal appearance, challenges with bleeding but tend to live a normal life

28
Q

what sex is affected by haemophilia

A

males

29
Q

what sex are the carriers of haemophilia

A

females

30
Q

what is the severe level of haemophila

A

less than 0.02iu/ml

2%

31
Q

what are the ranges of severe to moderate to mild to carriers of haemophilia

A

Severe <0.02iu/ml
Moderate 0.02-0.09 iu/ml
Mild 0.1 – 0.4 iu/ml
Carriers <0.5 iu/ml

32
Q

why is there such a large decrease between normal CF8 level to severe haemophilia

A

Lots of reserved capacity for F8

  • Take a while to see effect
  • Excessive capacity in kidneys
33
Q

what is the clinical effect of haemophilia A

A

small clotting problem, more bleeding when cut themselves

34
Q

treatment of severe and moderate haemophilia A

A

Require the use of recombinant factor VIII
- No other option/ substitution

Made by bacteria

  • Protein factor 8 purified and injected as needed
  • Depend on activity
35
Q

treatment of mild and carriers of haemophilia a

A

Majority respond to DDAVP

  • Vasopressin, like ADH
  • Release stuck F8 from endothelial cells (like glucose to haemoglobin)
  • Released due to competitive binding
  • Deals with mild bleeding problems

Very mild cases may only require oral tranexamic acid

  • Inhibitor of fibrinolysis
  • Slow down clot break down – important balance of 2 processes
  • Returns pt to normal
  • Slower and lower level of turnover of clots

Occasionally require F8
- E.g. Challenge to vascular system e.g. extraction

36
Q

treatment of haemophilia B

A

Do not response to DDAVP
- F9 is not bound to endothelial cells unlike F8

Prophylactic cover requires recombinant factor IX
- Only way to manage is with recombinant

37
Q

what type of inheritance is von WIllebrand disease

A

Autosomal dominant

  • Both sexes equally affected
  • Normal gene and spontaneous mutation or inherited
38
Q

what causes von Willebrand disease

A

Deficiency of von Willebrand factor with a reduction in factor VIII levels

Platelet and coagulation F8 problem

39
Q

what are the 3 types of von Willebrand disease

A

Type 1 - Dominant - Mild
Type 2 - Dominant - Mild
Type 3 - Recessive - Severe
- less likely - 2 parents pass on recessive gene

40
Q

what is the treatment for von willebrand disease

A

Majority of patients responds to DDAVP
- F8 platelet aggregation

Interacts with F8 in blood

  • Defective VW factor on platelet
  • Role of platelet in starting coagulation is affected
  • now ineffective

Very mild cases may only require oral tranexamic acid

41
Q

how are coagulation disorders treated throughout the UK

A

Common standardised treatment regimes for all patients.
- National management of Tx

Depend on level of risk that they pose

Treatment should be available locally.

  • Mild haemophilia – dentist can do all (sometime extractions in haemophilia centre)
  • Hospital visits reduced.
  • —-More convenient and simpler for pt to have locally managed
42
Q

in general how do severe and moderate coagulation disorder pt get treated

A

Majority of the treatment in the hospital except for prosthodontics.
- Denture by local – small bleeding risk

Treatment by GDP/PDS by arrangement

43
Q

in general how do mild and carriers of coagulation disorders get treated

A

Treatment shared with GDP/PDS.
- Extractions, LA

Patient reviewed at the hospital every 2 years.
- Check nothing needs changed, all fine

44
Q

5 types of dental treatment where special care would be required no matter the severity of coagulation disorder

A

administration of LA (esp ID nerve block)

extractions

minor oral surgery

periodontal surgery

biopsies

risk of blood loss

45
Q

safe LA injection sites for coagulopathy pt (3)

A

Buccal infiltration

Intraligamentary injections (space between tooth and gum)

Intra-papillary injections (space between the teeth)

46
Q

dangerous LA injection sites for coagulopathy pt (3)

A

Inferior alveolar nerve block

Lingual infiltration

Posterior superior (maxilla) nerve block

47
Q

can mild haemophiliac get LA injections by GDP

A

yes - if choice of LA and technique is correct

48
Q

what is the concern with extractions and dental surgeries for haemophiliacs

A

stopping bleeding

49
Q

how should a extraction/surgery be approached for a haemophiliac

A

Appropriate cover from Haemophilia Unit A
- Activity of F8 and 9 and inhibitors at basal and post injection level
- Work out the amount needed to be administered prior to extraction
- Haematologist prepare pt
traumatic treatment

Consider antibiotics

  • Infection will disturb clit
  • Routinely not used unless there is an infection

Observe for 2-3 hours after surgery (minimum requirement)

  • Initially bleeding is stopped by platelets
  • Takes a few hours for coagulation to build up fibrin clump over it

Comprehensive post-operative instructions

  • If start to bleed again
  • –Local treatment and potential more clotting factors needed
  • –Communication between GDP and haematologist
50
Q

how long should a severe and moderate coagulopathy pt be observed post surgery/extraction

A

Patients should be observed overnight following surgery

Risk of problem is higher with more they do

Take out of domestic environment to try and ensure low activity level
- Less BP and trauma to trigger bleeding

51
Q

how long should a mild and carrier pt be observed post surgery/extraction

A

2-3 hours after surgery

52
Q

what 9 possible parts of a coagulopathy pt comprehensive care team

A 
Patient
haematologist
physiotherapist
nurses
dental surgeon 
psycho-social workers
laboratory technicians
orthopaedic surgeon

A lot involved in managing these pt
Manage well - should have good quality of life

53
Q

what is thrombophilia

A

Increased risk of clots developing
- Opposite to haemophilia

Disproportionate clotting to fibrinolytic action
- Over producing clot or not enough fibrinolytic products

Can be relatively undetectable until make blood clot inappropriately

  • E.g. DVT leads to pulmonary embolism
  • Mild until certain set of circumstances causes them to become visible

Often an acquired condition superimposed on a genetic condition

Usually possible to find a cause for the clot

54
Q

what are 4 inherited syndromes that can lead to thrombophilia

A

Protein C deficiency

Protein S deficiency

Factor V Leiden

Antithrombin III deficiency

55
Q

what are 7 acquired syndromes that can lead to thrombophilia

A

Antiphospholipid syndrome
- Lupus anticoagulants (sub group of lupus)

Oral contraceptives

Surgery

Trauma

Cancer

Pregnancy

Immobilisation

risk of having high levels of clots

56
Q

how are acquired syndromes causing thrombophilia managed

A

managed by medications that will reduce the level of clots

57
Q

how are inherited syndromes of thrombophilia managed

A

broken down into individual groups and managed from there

58
Q

thrombocytopenia

A

redcued platelet numbers

59
Q

qualitative platelet disorders

A

normal platelet number but abnormal function

60
Q

thrombocytopenia

A

increased platelet numbers

61
Q

what are 3 platelet abnormalities

A

Thrombocytopenia

Qualitative disorders

Thrombocythemia

62
Q

what are causes of thrombocytopenia

A

Idiopathic

Drug related 
- Alcohol 
- Penicillins 
- Heparin 
secondary to lymphoproliferative disorders
63
Q

what is the effect on dental treatment of thrombocytopenia

A

dental treatment can proceed safely

providing the platelet count > 50 x10^9

64
Q

how can qualitative platelet disorders arise

A

inherited

65
Q

what are 3 rare qualitative platelet disorders

A

Bernard Soulier Syndrome

Hermansky Pudlak

Glanzmann’s thrombasthenia

66
Q

what are 4 reasons for acquired qualitative platelet disorders

A

Cirrhosis

Drugs

Alcohol

Cardiopulmonary bypass

67
Q

what is the treatment for thrombocythemia

A

Uncommon disease

Usually on aspirin to prevent clot formation

BDS2 BAMS (54 decks)

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