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eMCD - Hae > Haematology 8: Disorders of Haemostasis > Flashcards

Haematology 8: Disorders of Haemostasis Flashcards

1
Q

What is abnormal bleeding?

A

e. g minor bleeding
- easy bruising
- gum bleeding
- menorrhagia (in women)
- post part bleeding (In women)
- epistaxis (nosebleed)

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2
Q

Why do people have abnormal haemostasis?

- give 2 main reasons

A

due to:

  1. Lack of a specific Factor
  2. Defective function of a specific factor
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3
Q

Describe the genetic pattern of hemophilia.

A
  • sex linked

- recessive disorder

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4
Q

Give methods of treatment of bleeding disorders

A
  1. Failure of production/function:
    - -> replace missing factor/platelets
    - -> stop drugs
  2. Immune destruction:
    - -> Immunosuppression
    - -> Splenectomy for ITP
  3. Increased consumption:
    - -> Treat cause
    - -> replace
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5
Q

What are some disorders of primary homeostasis?

A
  • low platelet no.
    –> thrombocytopenia
    (bone marrow failure, accelerated clearance)
  • impaired function of platelets
  • -> due to hereditary absence of glycoproteins / storage granules
  • Von Willebrand disease
  • Vessel wall disorder
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6
Q

Give 3 mechanisms and causes of thrombocytopenia

A
  1. failure of platelet production by megakaryocytic
  2. shortened half life of platelets
  3. Increased pooling of platelets in enlarged spleen + shorted half life
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7
Q

Give three examples of hereditary platelet defects

what do they affect?

A
  • Glanzmann’s Thrombasthenia
  • -> problem with GPIIb/IIIa
  • Bernanrd Souller Syndrome
  • -> Problem with GP1B
  • Storage Pool Disease
  • -> problems with dense granules
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8
Q

What is the function of VWF in homeostasis?

A
  • binds to collagen + captures platelets

- stabilises factor 8

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9
Q

VWD is usually hereditary.

describe what is type (1+2+3)

A

Type 1 = VWF deficiency (but normal function)
Type 2 =VWF made (but abnormal function)
Type 3= VWF not made at all

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10
Q

Disorder of primary homeostasis due to vessel wall can be BOTH inherited + Acquired. Give examples of both

A

Inherited:

  • hereditary hemorrhagic telangiectasia
  • Ehlers Danlos syndrome

Acquired:

  • Scurvy
  • Steroid Therapy
  • Aging
  • Vasculitis
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11
Q

_______ might indicate thrombocytopenia –>

A

Thrombocytopenia gives rise to –> Petechiae

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12
Q

Give examples of tests for disorders of primary haemostasis

A
  • platelet count
  • bleeding time (platelet function analysis)
  • assays of VWF
  • clinical observation
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13
Q

Haemophilia = due to lack of factor __ and factor ___

This results in = impaired _____ ________

A

Haemophilia = due to lack of factor 8 and factor 9

This results in = Impaired thrombin generation

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14
Q

Measuring thrombin generation over time –> you can visualize ________

A

Measuring thrombin generation over time –> you can visualize process of coagulation

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15
Q

What is the role of the coagulation cascade?

A

to generate burst of thrombin which converts fibrinogen –> fibrin

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16
Q

How might disorders of coagulation arise?

A
  1. deficiency of coagulation factor production

2. Increased consumption

17
Q

Give examples of hereditary coagulation factor deficiency

A
  • factor 8 + 9 (hemophilia)
  • factor 2 (prothrombin) –> lethal
  • factor 11
  • factor 12 –> no excess bleeding
18
Q

Give examples of Acquired coagulation factor deficiency

A
  • liver disease –> coagulation factors = synthesized in liver
  • dilution
  • anticoagulant drugs e.g warfarin
19
Q

What is a hallmark of hemophilia?

A
  • Haemarthrosis
20
Q

List different tests or coagulation disorders

A
  • Screening tests (PT, APTT, Platelet Count)
  • Factor Assays
  • Test for inhibitors
21
Q

Note: but there are bleeding disorders not detected by routine clotting tests (e.g VWD, factor 13 deficiency, platelet disorders etc.)

A

-

22
Q

Disorders of fibrinolysis can be BOTH hereditary + acquired.
Give examples of both.

A

Hereditary:
- e.g Antiplasmin Deficiency

Acquired:

  • e.g Drugs
  • Disseminated intravascular coagulation
23
Q

Describe the genetic pattern of VWD

A
  • autosomal dominant
24
Q

Why might you have low thrombocytopenia?

A
  • bone marrow failure (leukemia, B12 deficiency)

- accelerated clearance

25
Q

most coagulation factors are produced in the _____

A

liver

26
Q

Hemophilia is dominant/recessive.

it is X linked / Y linked

A

recessive

X- linked

27
Q

VWD = autosomal dominant / recessive

A

dominant

28
Q

how would you treat abnormal haemostasis if there was failure of production or function?

A
  • replace missing factor
    (factor replacement therapy e.g Prothrombin Complex Concentrates factors - PCC)
  • if person is on drug –> stop drug
29
Q

How is DDAVP used as a homeostatic treatment?

A
  • binds to V2 receptors
    causes exocytosis of VWF and Factor 8

–> used to treat hemophilia + VWD

30
Q

How is Tranexamic acid used as a homeostatic treatment?

A
  • inhibits fibrinolysis
31
Q

How would you ensure bleeding test = conducted accurately?

A
  • Standardised incision on patient’s arm
  • Use pressure cuff
  • Accurate time measuring device

eMCD - Hae (11 decks)

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