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Haematology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

What is Haemophilia A?

A

Deficiency of factor 8

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2
Q

What is Haemophilia B?

A

Deficiency of factor 9

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3
Q

What is the most common inherited bleeding disorder?

A

Von Willebrand disease

Haemophilia A

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4
Q

What is the mode of inheritence of haemophilia A and B?

A

X linked recessive

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5
Q

How does heamophilia A and B present?

A

Haemarthrosis/bleeding into joint

  • Tense swollen knee without injury

Haematuria

Muscle haematoma

CNS bleeding

Retroperitoneal bleeding

Post surgical bleeding

Secondary arthritis

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6
Q

What investigations are used in haemophilia A and B diagnosis?

A

Factor 8/9 Assay

Increased APTT

  • As intrinsic pathway is affected

Normal PT, platelets and vWF

Genetic analysis

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7
Q

How is haemophilia A and B managed?

A

Haemophilia A

  • Purified factor 8
  • Desmopressin, which increases factor 8 levels

Haemophilia B

  • Factor 9 injections

Tranexamic Acid

Gene therapy

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8
Q

What is the prophylactic management of haemophilia A and B?

A

Splints

Physiotherapy

Analgesia

Synovectomy

Joint replacement

Regular coagulation factor injections

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9
Q

What does Residual Coagulation Factor Activity assess?

A

Severity of bleeding

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10
Q

What is mild haemophilia?

A

5-30% residual coagulation factor activity

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11
Q

What is moderate haemophilia?

A

1-5% residual coagulation factor activity

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12
Q

What is severe haemophilia?

A

<1% residual coagulation factor activity

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13
Q

Give complications of haemophilia A and B

A

Synovitis

Chronic haemophilic arthropathy, arthritis caused by bleeding into joints

Compartment syndrome

Other sequelae of bleeding, such as stroke

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14
Q

What is type 1 Von Willebrand disease

A

Partial reduction in vWF

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15
Q

What is type 2 Von Willebrand disease?

A

Abnormal form of vWF

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16
Q

What is type 3 Von Willebrand disease?

A

Total lack of vWF, autosomal recessive

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17
Q

What mode of inheritence is von willebrand disease?

A

Various subtypes, however the most common is autosomal dominant

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18
Q

How does von willebrand disease present?

A

Mucosal/capillary bleeding

Easy bruising

19
Q

What investigations are used in von willebrand disease?

A

Low or abnormal vWF

Slightly increased APTT

Normal PT

Normal platelets

Prolonged bleeding time

20
Q

How is von willebrand disease managed?

A

vWF concentrate

Tranexamic acid for mild bleeding

DDVAP (Desmopressin) infusions

  • Induces release of vwf
21
Q

What is autoimmune thrombocytopenic purpura (ITP)?

A

Thrombocytopenia characterised by antibodies against the antigens on the platelet surface, resulting in removal via the reticuloendothelial system

22
Q

What age group does ITP affect?

A

Middle aged women and children

23
Q

How does ITP present?

A

Recurrent epistaxis

Ecchymosis/bruising

Gingival bleeding

Menorrhagia

Recent URTI

24
Q

What investigations are used in ITP?

A

FBC

  • Thrombocytonpenia

Blood film

Bone marrow biopsy

25
Q

How is ITP managed?

A

Emergency

  • Platelet transfusion
  • IV methylprednisilone
  • IV immunoglobulin

If platelet count is over 30

  • Observation

If platelet count is less than 30

  • Oral prednisilone, first line
  • IV immunoglobulin
  • If resistant, consider splenectomy
26
Q

What is thrombotic thrombocytopenic purpura?

A

Deficiency of enzyme ADAMTS13 which breakdowns large multimers of von Willebrand’s factor

27
Q

What causes thrombotic thrombocytopenic purpura?

A

Post-infection

Pregnancy

Drugs

  • Ciclosporin
  • Oral contraceptive pill
  • Penicillin
  • Clopidogrel
  • Aciclovir

Tumours

SLE

HIV

28
Q

What are features of thrombotic thrombocytopenic purpura?

A

Rare, typically adult females

Fever

Fluctuating neuro signs

Microangiopathic haemolytic anaemia

Thrombocytopenia

Renal failure

29
Q

What is Wiskott-Aldrich syndrome?

A

Primary X linked recessive immunodeficiency due to a combined B and T cell dysfunction, caused by mutation in the WASP gene and characterised by recurrent bacterial infections, eczrma and thrombocytopaenia

30
Q

At what platelet level is autoimmune thrombocytopenic purpura treated?

A

Only treat if platelets <30x10^9/l

31
Q

What coagulation factors are affected with heparin?

A

Affects activation of 2 9 10 11

32
Q

What factors are affected with warfarin?

A

Affects synthesis of 2 7 9 10

33
Q

What coagulation factors are affected by DIC?

A

1 2 5 8 11

34
Q

What coagulation factors are affected in liver disease?

A

1 2 5 7 9 10 11

35
Q

What are the blood clotting results in haemophilia?

A

APTT inncreased

PT normal

Bleeding time normal

36
Q

Describe the blood clotting results in vWd

A

APTT increased

PT normal

Bleeding time increased

37
Q

Describe the blood clotting results in vitamin K deficiency

A

APTT increased

PT increased

Bleeding time normal

38
Q

Give causes of thrombocytopenia

A

DIC

Haemotological malignancy

Aspirin

Diuretics

Alcohol

Liver disease

Hypersplenism

EBV, HIV, Hepatitis

Pregnancy

SLE, antiphospholipid syndrome

Vitamin B12 deficiency

39
Q

What is the mode of action of LMWH?

A

Activates antithrombin III and forms a complex that inhibits factor Xa

40
Q

What monitoring is used in LMWH?

A

Anti factor Xa, although routine monitoring is not necessary

41
Q

What monitoring is used in standard heparin?

A

Increased APTT

42
Q

What is the mode of action of standard heparin?

A

Activates antithrombin III and forms a complex that inhibits thrombin, factors Xa, IXa, Xia and XIIa

43
Q

How is heparin induced thrombocytopenia managed?

A

Switch to a direct thrombin inhibitor, such as argatroban

Haematology (7 decks)

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