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Haematology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

What is Disseminated Intravascular Coagulation (DIC)?

A

A condition where the inappropriate activation of the clotting cascade results in thrombosis formation and leads to depletion of clotting factors and platelets.

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2
Q

What are some causes of DIC?

A
  • Secondary to Sepsis!
  • Obstetric Emergencies
  • Malignancy (Acute Promyelocytic Leukaemia)
  • Hypovolaemic shock
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3
Q

What two methods result in activation of coagulation in DIC?

A

Either
- Release of procoagulant material (e.g. Tissue factor)
OR
- Via cytokine pathways as part of the inflammatory response.

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4
Q

What is generated to commence coagulation in DIC?

A

Widespread generation of fibrin and deposition in blood vessels, leading to thrombosis and multiorgan failure.

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5
Q

What happens to the Platelets and Coagulation factors in DIC?

A

The widespread coagulation uses them up.

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6
Q

What else is activated other than coagulation in DIC leading to the production of FDPs and D-Dimers.

A

There is Secondary Activation of Fibrinolysis.

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7
Q

What are FDPs and D-Dimers?

A

Fibrin Degradation Products and D-Dimers are both little chunks of broken up fibrin.
- Only get D-dimers from the breakdown of Clots.

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8
Q

What are the resulting consequences of coagulation activation and secondary action of Fibrinolysis?

A

A mixture of, Initial Thrombosis, Followed by a bleeding tendency due to consumption of coagulation factors and dysregulated fibrinolytic activation.

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9
Q

What does the Microvascular Thrombus formation manifest as?

A

End Organ Failure.

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10
Q

What does the Clotting factor consumption manifest as clinically?

A

Bruising, Purpura, Generalised Bleeding.

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11
Q

What is the Typical Clinical Presentation of DIC?

A

Patient is often acutely unwell and shocked.
Clinical presentation varies from no bleeding, to Profound haemostatic failure with widespread haemorrhage.

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12
Q

What symptoms would a patient with DIC exhibit?

A

Epistaxis, Gingival bleeding, Haematuria, Bleeding from cannula sites.
- Fever
- Confusion
- Potential Coma

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13
Q

What signs are common in DIC?

A
  • Petechiae
  • Bruising
  • Confusion
  • Hypotension.
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14
Q

What would the PT, APTT and TT usually show in DIC?

A

Usually very prolonged.

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15
Q

What is seen on Blood tests in DIC?

A

High levels of FDPs, including D-Dimers.
Severe Thrombocytopenia.
Blood film may show fragmented RBCs.

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16
Q

What is the management in DIC?

A

Treat underlying cause.
Supportive care
- Transfusions of platelets or clotting factors.
- Anticoagulation therapy may be necessary.

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17
Q

What is Haemophilia?

A

Hereditary disorder in which abnormally prolonged bleeding recurs episodically at one or a few sites on each occasion.

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18
Q

What is the genetic inheritance pattern in Haemophilia?

A

X-Linked recessive

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19
Q

What is Haemophilia A a deficiency of?

A

Factor VIII
(5x more common than Haemophilia B)

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20
Q

What is Haemophilia B a deficiency of?

A

Factor IX

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21
Q

Is there an abnormality of primary haemostasis in Haemophilia?

A

No

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22
Q

Where does bleeding usually occur in Haemophilia?

A

Bleeding from medium to large blood vessels, most commonly into joints.

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23
Q

What determines the severity of Haemophilia in Families?

A

Depends on Factor VIII/IX level involved.

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24
Q

How do patients with Mild Haemophilia usually present ?

A

Bleeding is usually only associated with injury or surgery so diagnosis is often made quite late in life.

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25
Q

What are some clinical features of severe haemophilia?

A
  • Recurrent Haemarthroses
  • Recurrent soft tissue bleeding. (Bruising in toddlers)
  • Prolonged bleeding after dental extractions, surgeries etc..
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26
Q

What is seen on PT, APTT and TT in patients with Haemophillia?

A

An Isolated prolonged APTT.

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27
Q

What kind of test can be done to isolate the factor deficiency in Haemophilia?

A

Coagulation factor assays.

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28
Q

What can be used in Acute episodes of bleeding or prevention of excessive bleeding during surgery in patients with Haemophilia?

A
  • Desmopressin For minor bleeds (Stimulates release of VWF)
  • Infusions of Affected factor (VIII or IX) for major bleeds.
  • Antifibrinolytics (Tranexamic Acid) are useful for bleeding wounds.
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29
Q

When should Antifibrinolytics (Tranexamic Acid) be avoided in Haemophilia Tx?

A

In muscle Haematomas, Haemarthrosis and Urinary bleeding as they can lead to fibrosis.

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30
Q

For Severe Haemophilia, Deficient factors can be replaced via IV infusions either as prophylaxis or in response to bleeding. What is a potential complication of this treatment?

A

The formation of antibodies against the clotting factor resulting in Tx becoming ineffective.

31
Q

What is Haemophilia with inhibitors? And how is it Treated?

A

1/3 of Boys w Haemophilia A will develop an inhibitor to factor VIII following Infusions.
- Treatment should be directed by a specialist centre.

32
Q

What immunisations are important in Haemophilia?

A

Hep B

33
Q

What is Von-Willebrand’s Disease?

A

An inherited bleeding disorder characterised by a reduced quantity or function of VWF.

34
Q

What is the primary cause of VWD?

A

A genetic mutation that results in deficiency or dysfunction of VWF.
- VWF gene located on Chromosome 12 (lots of mutations been identified)

35
Q

What role does VWF play in Haemostasis?

A

Has a critical role as an adhesive protein in the platelet vessel wall interaction. The absence of this leads to impaired platelet adhesion and failure to create the platelet plug.

36
Q

What deficiency can reduced VWF levels also lead to?

A

Deficiency in Factor VIII as it is not protected from premature degradation.

37
Q

What clinical features are seen in Von-Willebrand’s Disease?

A

Range from Mild to more severe bleeding:
- Excess or prolonged bleeding from wounds.
- Excess or prolonged bleeding post-op.
- Easy Bruising
- Menorrhagia.
- Epistaxis.
- GI bleeding.

38
Q

What would an FBC show in VWD?

A

May be normal or show microcytic anaemia.
NORMAL platelet count.

39
Q

What would APTT, PT and TT show in VWD?

A

APPT may be normal or prolonged if factor VIII deficiency is present.
PT will be normal.

40
Q

What can be tested for in VWD investigations?

A

VWF antigen test.
Factor VIII clotting activity test.
Specialist VWF testing e.g. VWF multimer test.

41
Q

What is the management of VWD?

A

Depends on Severity.
- May involve Desmopressin.
- Plasma-derived Factor VIII concentrated containing intact VWF are mainstay of replacement therapy.
(Used to Tx bleeding or in surgery, or those who do not respond adequately to Desmopressin)

42
Q

What is Immune thrombocytopenic purpura?

A

A syndrome where there is a decreased number of circulating platelets which leads to a bleeding tendency.

43
Q

How can ITP occur?

A

May occur in isolation (primary ITP) or associated with other disorders (secondary)

44
Q

What are some causes of Secondary ITP?

A
  • Other autoimmune disorders (Anti-Phospholipid antibody syndrome and SLE)
  • Viral Infections (CMV, VZV, Hep C and HIV)
  • Infection with H.Pylori
  • Medications
  • Lymphoproliferative disorders
45
Q

What is the Pathophysiology of ITP?

A

Caused by a type 2 hypersensitivity reaction:
- It is caused by the production of antibodies that target and destroy platelets.
- This can happen spontaneously, or it can be triggered by something, such as a viral infection.

46
Q

What sort of Symptoms can ITP cause in otherwise healthy patients?

A
  • Petechiae
  • Ecchymoses
  • Purpura
  • Bleeding from venepuncture sites (nose, etc..)
47
Q

What is Ecchymoses?

A

A bruise

48
Q

What are purpura?

A

Purple spots or patches on your skin or mucous membranes.

49
Q

What age does ITP usually present?

A

Under 10.
(often with a recent history of recent viral illness)

50
Q

What would a blood test show in ITP?

A

Isolated Thrombocytopenia.
(no other findings)
- If something appears abnormal a bone marrow examination would likely be next course of action.

51
Q

What is the management of ITP?

A

Usually no TX required and patients are monitored until platelets return to normal.
(~70% of patients will return to normal within 3 months)

52
Q

When would intervention be needed in ITP?
- What intervention would be indicated?

A

If the patient is actively bleeding or severe thrombocytopenia (Platelets <10)
- Prednisolone
- IV Immunogobulins
- Blood transfusions if required
- Platelet Transfusions only work temporarily.

53
Q

What is thrombotic Thrombocytopenia Purpura (TTP)?

A

Rare form of thrombotic microangiopathic haemolysis, thrombocytopenia and neurological abnormalities.

54
Q

What is the aetiology of TTP?

A

Occurs due to a deficiency of metalloproteinase ADAMTS13.
- Can be a hereditary congenital mutation or due to autoimmune inhibition.

55
Q

What sorts of triggers is TTP associated with?

A
  • Medications
  • AIDS
  • Malignancy
56
Q

What is the classical pentad for TTP presentation?

A
  • Fever
  • Microangiopathic Haemolytic anaemia (MAHA)
  • Thrombocytopenic purpura
  • CNS involvement: Headache, Confusion, Seizures.
  • AKI
57
Q

What would a FBC of a patient with TTP show?

A

Normocytic anaemia (secondary to haemolysis), Thrombocytopenia, and possibly a raised neutrophil count.

58
Q

What would U&E show in a patient with TTP?

A

Raised Urea and Creatinine.

59
Q

What would be seen on a blood film of a patient with TTP?

A
  • Schistocytes (consistent with haemolysis).
  • An elevated Retic count.
60
Q

Which other common bloods would be elevated in TTP?

A

LFT
LDH
D-dimer
Indirect bilirubin

Haptoglobins would be low.

61
Q

What helps differentiate between DIC and TTP?

A

Coagulation screen in TTP will be normal.
(Prolonged in DIC with low plasma fibrinogen)

62
Q

What type of Assay is Diagnostic in TTP?

A

ADAMST13 assay
- Low ADAMST13 activity is diagnostic.

63
Q

What would be seen on Urinalysis of a patient with TTP?

A

Proteinuria and Microscopic Haematuria.

64
Q

What is the management of TTP?

A
  • Fresh frozen plasma
  • Plasma exchange
  • High-dose steroids, low-dose aspirin and rituximab can also be given.
  • Splenectomy may be used to Tx patients who do not respond to plasma exchange, or who relapse chronically.
65
Q

What does Fresh frozen plasma contain in the context of TTP treatment?

A

Contains VWF-cleaving protease and complement components.

66
Q

What does plasma exchange do in TTP?

A

Removes antibodies and toxins associated with the pathogenesis of the disease.

67
Q

What is the first thing you should do on finding a major haemorrhage patient?

A

Trigger the major blood loss protocol.

68
Q

What is the second step in the management of Massive Haemorrhage?

A

Take baseline blood samples before transfusion for:
- FBC, group and save, clotting screen.

69
Q

What should be given to a patient with a massive haemorrhage as a result of trauma, who has presented within 3 hrs of the injury?

A

Tranexamic acid 1g bolus over 10 mins followed by IV infusion of 1g over 8 hrs.

70
Q

What needs to be given in Massive Haemorrhage if Hb is Falling?

A

Red cells; Helps maintain tissue oxygenation.

71
Q

What needs to be given in Massive Haemorrhage if PT ratio is >1.5?

A

FFP 15-20ml/Kg
- Replaces coagulation factors and helps to maintain coagulation close to normal.

72
Q

What should be given in Massive Haemorrhage if Fibrinogen is <1.5g/L?

A

Cryoprecipitate (2 pools)
- Replaces Fibrinogen.

73
Q

What should be given in Massive Haemorrhage if Platelets are <75x10^9/L?

A

Give Platelets 1 ATD.
- Helps correct thrombocytopenia.

Haematology (10 decks)

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