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HAEMATOLOGY COPY > BLEEDING DISORDERS AND THROMBOSIS > Flashcards

BLEEDING DISORDERS AND THROMBOSIS Flashcards

1
Q

In a patient with a bleeding disorder where the mucous membranes are most affected, what is the likely underlying pathology?

A

Platelet defects
Von Willebrand disease

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2
Q

In a patient with a bleeding disorder where the bleeding happens into joint and muscles, what is the likely underlying pathology?

A

Coagulation factor deficiencies

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3
Q

What are the common features of a history of someone with a bleeding disorder?

A

Prolonged epistaxis (nosebleed)
Cutaneous haemorrhage or bruising with minimal or no trauma
Prolonged bleeding from trivial wounds
Oral cavity bleeding
Spontaneous gastrointestinal bleeding
Menorrhagia not associated with structural lesions of the uterus

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4
Q

What are the common acquired causes of a coagulopathy?

A

Vitamin K deficiency
Disseminated Intravascular Coagulation (DIC)
Liver disease

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5
Q

In the coagulation cascade, what is the purpose of factor VIII?

A

When converted into VIIIa, it enables the conversion of factor X into Xa. IXa must also be present for this to happen.

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6
Q

What are the two pathways of the coagulation cascade?

A

Contact activation (intrinsic) pathway
Tissue factor (extrinsic) pathway

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7
Q

What are the factors that make up the intrinsic (contact activation) pathway of the coagulation cascade?

A

XII - XIIa
XI - XIa
IX - IXa
VIII - VIIIa

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8
Q

What are the factors that make up the extrinsic (tissue factor) pathway of the coagulation cascade?

A

VII - VIIa
X - Xa
Tissue factor (III)

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9
Q

What are the factors that make up the common pathway of the coagulation cascade?

A

II (Prothrombin)
IIa (Thrombin)
V - Va
I (Fibrinogen)
Ia (Fibrin)
XIII - XIIIa

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10
Q

What does the activated partial thromboplastin time (APTT) represent?

A

Performance indicator of the efficacy of both the intrinsic (contact activation) pathway and the common coagulation pathway.

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11
Q

What does the prothrombin time (PT) represent?

A

Measure of the extrinsic pathway of coagulation.

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12
Q

What factor is deficient in haemophilia A?

A

VIII

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13
Q

What factor is deficient is haemophilia B?

A

IX

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14
Q

What is the prevalence of haemophilia A among males?

A

1 in 5000

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15
Q

What is the prevalence of haemophilia B among males?

A

1 in 25000

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16
Q

Below what percentage of factor VIII or IX are patients considered to have severe haemophilia?

A

Less than 1%

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17
Q

Within what range, as a percentage of factor VIII or IX, are patients considered to have moderate haemophilia?

A

1-5%

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18
Q

Above what percentage of factor VIII or IX are patients considered to have mild haemophilia?

A

> 5%

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19
Q

What is the standard treatment for patients with mild haemophilia A?

A

Desmopressin or clotting factors

20
Q

What is the treatment for patients with moderate or severe haemophilia A?

A

Clotting factor VIII injections

21
Q

What commonly used drugs must those with bleeding disorders avoid?

A

NSAIDs and aspirin

22
Q

What is another term for haemophilia B?

A

Christmas disease

23
Q

What is the role of von Willebrand factor?

A

adhesive protein in platelet vessel wall interaction,

Protects factor VIII in the circulation

24
Q

What are the clinical features of von Willebrand disease?

A

Mucocutaneous bleeding as a result of abnormal platelet function.
Menorrhagia is common.

25
Q

Types of Von Willebrand’s disease?

A

• Type 1: partial quantitative deficiency of VWF; usually inherited as an autosomal dominant.

• Type 2: qualitative abnormality of VWF; also usually inherited as an autosomal dominant.

• Type 3: virtually complete deficiency of VWF: recessively inherited.

Many subtypes of VWD are described, particularly type 2 variants,

26
Q

What is the pattern of inheritance of von Willebrand disease?

A

Autosomal dominant

27
Q

How is von Willebrand disease treated?

A

Desmopressin

plasma-derived factor VIII concentrates that contain intact VWF (mainstay of replacement therapy)

recombinant VWF is becoming available

(later two to cover surgery, bleeding episodes or in type 3)

28
Q

Haemostasis defect(s) in liver disease?

A

• Vitamin K deficiency (d/t cholestasis)

• Reduced synthesis. Reduced synthesis of coagulation factors (including natural anticoagulant proteins)

• Thrombocytopenia (from hypersplenism <- portal hypertension, or from folic acid deficiency)

• Functional abnormalities (of platelet/ fibrinogen)

• DIC (in acute hepatic failure)

29
Q

What are the three common acquired bleeding disorders?

A

Vitamin K deficiency
Liver disease
Disseminated intravascular coagulation

30
Q

What is disseminated intravascular coagulation?

A
  • widespread activation of clotting cascade
  • results in the formation of blood clots in the small blood vessels throughout the body. This leads to compromise of tissue blood flow and can ultimately lead to multiple organ damage.
  • as the coagulation process consumes clotting factors and platelets, normal clotting is disrupted and severe bleeding can occur
31
Q

What conditions can lead to DIC?

A

Cancer - both solid and blood (particularly acute promyelocytic leukemia)
Sepsis
Trauma, burns, surgery
Liver Disease
Severe allergic or toxic reaction - eg snake venom
Transfusion reaction

Obstetric issues - pre-eclampsia, amniotic fluid embolism

32
Q

What investigations might be ordered to further assess someone with suspected disseminated intravascular coagulation? For each state what might be found in a positive result.

A

PT, aPTT, TT - all increased
Fibrinogen - low
Plt - v low
D-dimer ++
Blood film: schistocytes

33
Q

How is disseminated intravascular coagulation treated?

A

Treat cause

FFP, platelets, cryoprecipitate, RBCs
NO Heparin

34
Q

How does liver disease lead to a bleeding disorder?

A

Loss of synthetic function which results in reduced amounts of proteins used in the coagulation cascade. Coagulopathy usually happens in conjunction with thrombocytopenia.

35
Q

What is thromobocytopenia?

A

Low platelet count

36
Q

How is vitamin K involved in the coagulation cascade?

A

Vitamin K is involved in the post-translational modification of factors II, VII, IX and X.
It is also involved in activated protein C.

37
Q

What are the risk factors for venous thromboembolism?

A

Age
Immobilization and paresis
Surgery and trauma
Malignancy
Pregancy and the puerperium (post-delivery)
Combined oral contraceptive pill
Hormone replacement therapy
Inherited thrombophilias
Antiphospholid antibodies
Raised coagulation factors
Family history
Serious illness
Obesity
Varicose veins
Smoking

38
Q

How is the diagnosis of a DVT or PE excluded?

A

Ultrasound
D-dimer

39
Q

What are the inherited thrombophilias?

A

Antithrombin deficiency
Protein C deficiency
Protein S deficiency
FV Leiden
Increased levels of prothrombin (G20210A)

40
Q

How does antithrombin work?

A

Most of the coagulation proteins are serine proteases. Antithrombin is a serine protease inhibitor. It works mainly to neutralise thrombin (factor II), but it also has some effect on Xa.

41
Q

How do proteins C and S work as natural anticoagulants?

A

Activated protein C cleaves the two co-factors in the intrinsic coagulation pathway (V and VIII). Protein S is the cofactor to protein C. They are both vitamin K dependent proteins.

42
Q

How is protein C activated as a natural anticoagulant?

A

It is activated by thrombin in the presence of an endothelial cofactor, thrombomodulin.

43
Q

Are heterozygotes of antithrombin, protein C or protein S deficiency affected?

A

Yes, they have 50% of the levels and are therefore at risk. Therefore it is considered autosomal dominant.

44
Q

What is FV Leiden disease?

A

This is a disease where there is resistance to protein C, a natural anticoagulant. It is as result of a mutation in the gene for factor V.

45
Q

What is the prevalence of FV Leiden disease among DVT patients?

A

20%

46
Q

How are venous thrombolembolisms treated?

A

Anticoagulation with heparin (SC, OD) followed by warfarin for 6 months if there has been no identified cause or lifelong if this is a recurring DVT/PE. Target INR is 2-3.

HAEMATOLOGY COPY (12 decks)

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