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Module 5 - Haematology > Inherited bleeding disorders > Flashcards

Inherited bleeding disorders Flashcards

1
Q

What may an inherited bleeding disorder affect?

A
  1. COAGULATION CASCADE (too little clot = hemophilia, too much clot = thrombophilia).
  2. **PLATELETS* number or function
  3. COMBINED DEFICIENCY
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1
Q

What is an inherited bleeding disorder?

A

An acquired defect which affects the coagulation of the blood.

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2
Q

Name 4 disorders that REDUCE COAGULATION FACTORS.

A
  • Factor VIII (8) deficiency = Hemophilia/ Hemophilia A.
  • Factor IX (9) deficiency = Christmas disease/ Hemophilia B.
  • Von Willebrand’s disease = Reduced factor VIII level + reduced platelet aggregation.
  • Factor XI (11) deficiency = common in Ashkenazy Jews.
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3
Q

What is deficient in hemophilia/ hemophilia A?

A

Factor VIII

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4
Q

What is deficient in hemophilia B/ Christmas disease?

A

Factor IX

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5
Q

What factor is deficient in von Willebrand’s disease?

A

Factor VIII + reduced platelet aggregation

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6
Q

What factor is commonly deficient is Ashkenazy Jews?

A

Factor XI.

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7
Q

How is Hemophilia A and B inherited? Who is most affected?

A
  • SEX LINKED (x chromosome) RECESSIVE.
  • females are CARRIERS, males are AFFECTED.
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8
Q

What determines the SEVERITY of hemophilia A and B?

A

The AMOUNT of factor produced (the less the more severe).

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9
Q

What is the NORMAL amount of clotting factor produced? What about carriers, mild, moderate and severe?

A
  • Normal: 1iu/mL
  • Carrier: more than 50% activity, <0.5iu/mL.
  • Mild: 10-40% activity , 0.1-0.4iu/mL
  • Moderate: 2-9% activity, 0.02-0.09iu/mL
  • Severe: less than 2% activity, <0.02iu/mL.
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10
Q

What is the management of moderate and severe hemophilia A?

A
  • RECOMBINANT FACTOR VIII.
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11
Q

What is the management of carrier and mild hemophilia A?

A
  • DDAVP/Desmopressin
  • Tranexamic acid if very mild.
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12
Q

What is the function of DDAVP?

A
  • Desmopressin
  • releases factor VIII bound to endothelial cells
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13
Q

What is the function of tranexamic acid?

A

INHIBITS FIBRONOLYSIS (keeps any clot that is formed for longer).

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14
Q

What is the managment of Hemophilia B? Why?

A
  • Carrier, mild, moderate severe ALL REQUIRE RECOMBINANT FACTOR IX.
  • NO ALTERNATIVE TREATMENT AVAILABLE.
    (DDAVP not effective as factor IX not bound to endothelial cells. Tranexamic acid not enough by itself.)
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15
Q

What are coagulation factor inhibitors?

A

ANTIBODIES which develop to RECOMBINANT FACTOR VIII AND IX.

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16
Q

How can coagulation factor inhibitors be minimized?

A

plan to give AS FEW EPISODES OF FACTOR CONCETRATE AS POSSIBLE

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17
Q

How is von Willebrand’s Disease inherited? Who is most affected?

A

AUTOSOMAL DOMINANT - BOTH SEXES EQUALLY AFFECTED.

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18
Q

What does von Willebrand disease cause? (ex. what deficiency).

A
  • Reduced Factor VIII.
  • Deficiency of von Willebrand factor/ defective vW factor on platelets

–> POOR CLOT ACTIVATION BY PLATELETS.

19
Q

What are the 3 types of vW disease? Which is most common?

A

o Type 1: Dominant Mild
o Type 2: Dominant Mild.
o Type 3: Recessive Severe.

  • Type 1 and 2 most common.
20
Q

What is the management of moderate and severe vW disease?

A

DDAVP enough for most.

21
Q

What is the management of mild/ carrier vW disease?

A

Tranexamic acid may be enough.

22
Q

What determines the bleeding risk in a patient with Hemophilia (A/B) and vW disease?

A

BASELINE FACTOR ACTIVITY, the HIGHER the factor the LOWER the risk.

23
Q

Which local anesthetics are safe in hemophilia patients? Which are not?

A

SAFE:
- intrapapillary.
- intraligamentary.
- buccal infiltration.

UNSAFE:
- lingual infiltration.
- IANB
- Posterior superior nerve block.

24
Q

How long post-extraction must a patient with carrier/mild hemophilia be observed?

A

2-3 hours after surgery.

25
Q

How long post-extraction must a patient with moderate/severe hemophilia be observed?

A

Must be observed OVERNIGHT.

26
Q

A patient with mild/carrier hemophilia requires restorative dentistry. Where will this be undertaken?

A
  • Prosthodontics/ procedures without bleeding or LA –> GDP.
  • LA: buccal, intraligamentary, intrapapillary –> GDP
  • LA: IANB, lingual infiltration, posterior superior nerve block –> HOSPITAL
27
Q

A patient with mild/carrier hemophilia requires an extraction/surgery. Where will this be undertaken?

A

HOSPITAL.

  • extractions, minoral oral surgery, biopsies, periodontal surgery.
28
Q

Name 4 procedures for which special care is requiered in Hemophilia patients.

A
  • biopsies.
  • extractions.
  • minor oral surgery.
  • periodontal surgery.
29
Q

A dentate patient with moderate/ severe hemophilia requires treatment. Where will this be undertaken?

A

Treatment in hospital or by arrangment with hemophilia unit.

30
Q

A edentulous patient with moderate/ severe hemophilia requires treatment. Where will this be undertaken?

A

Can be carried out in GDP

31
Q

Which coagulation factor deficiency condition is most common (rank them).

A
  1. vW.
  2. Hemophilia A
  3. Hemophilia B/ Factor XI.
32
Q

What is thrombophilia?

A

INCREASED risk of clots developing.

33
Q

How does on develop thrombophilia? (environmental? genetic?)

A
  • GENETIC tendency which is ENHANCED by local factors/ acquired problem:

ex. immobility from long flight, surgery, smoking, pregnancy.

34
Q

What is a great risk with thrombophilia?

A

If clot EMBOLIZES it can lead to BLOCKAGE OF A MAJOR VESSEL in the heart and/or lungs causing PULMONARY THROMBOEMBOLISM.

35
Q

Is thrombophilia inherited or acquired? Give examples.

A
  • Inherited Hypercoagulation: protein S deficiency, protein C deficiency, factor V leiden, antithrombin III deficiency.
  • Acquired Hypercoagulation: oral contraceptives, pregnancy, surgery, trauma, cancer, immobilization, antiphospholipid syndrome.
36
Q

Name 3 platelet disorders that INCREASE BLEEDING.

A
  1. Thrombocytopenia (reduced number of platelets).
  2. Qualitative diseases (normal platelet count but abnormal function)
  3. Thrombocythemia (increased number of abnormal platelets).
37
Q

What are 3 causes of thrombocytopenia?

A
  1. Idiopathic.
  2. Drug related (penicillin based, alcohol, heparin).
  3. Secondary to lymphoproliferative disorder (leukaemia or myelodysplasia).
38
Q

What is an issue in patients with lymphoproliferative disorders?

A
  • Myelodysplasia, Leukemia.

THROMBOCYTOPENIA.

39
Q

Are Qualitative Disorders Inherited or Acquired? Give examples.

A

BOTH.

Inherited: bernard soulier syndrome, hermansky pudlak, glanzmann’s thromboasthenia. RARE.

Acquired: Cirrhosis, alcohol, drugs, cardiopulmonary bypass.

40
Q

What is the usual cause of thrombocythemia?

A

Preneoplastic condition.

41
Q

What drug are thrombocythemia patients often on?

A

aspirin to prevent clot formation.

42
Q

What platelet count in necessary to treat a patient with a platelet disorder in primary care?

A

GREATER than 100 x 10^9/ L

43
Q

What platelet count in necessary to treat a patient with a platelet disorder in hospital?

A

GREATER than 50 x 10^9/L

44
Q

What procedures can a patient with a 100 x10^9/L platelet disorder undertake?

A

Hygiene therapy, prosthodontics, endodontics, orthodontics, restorative dentistry are FINE.

Simple extractions are FINE, ask advice form a SPECIALIST FOR SURGICAL.

45
Q

When is special care required for a patient with thrombocythemia?

A

platelet count above 500x10^9/L.

for extractions, surgery, biopsies, periodontal surgery.

Module 5 - Haematology (7 decks)

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