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103 Theme 2 - Blood > Bleeding disorders > Flashcards

Bleeding disorders Flashcards

1
Q

What are the 3 causes of bleeding (and any subtypes)?

A

Vascular disorders

Platelet disorders:

  • Thrombocytopenia
  • Defective function

Defective coagulation:

  • Inherited
  • Acquired
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2
Q

What are the 2 different patterns of bleeding?

A

Vascular and platelet causes - Bleeding into mucous membranes and skin

Coagulation disorders - Bleeding into joints and soft tissues

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3
Q

What causes vascular bleeding?

A

Problems with vessel wall

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4
Q

What are the 2 types of vascular bleeding and name some examples

A

Inherited:

  • Hereditary haemorrhagic telangiectasia
  • Ehlers-Danlos syndrome

Acquired:

  • Scurvy, steroids, senile
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5
Q

What condition is this and what type of bleeding is it?

A

Hereditary haemorrhagic telangiectasia

Also known as Osler-Weber-Rendu

Vascular bleeding - inherited

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6
Q

What is thrombocytopaenia and what is the reference range?

A

Low platelets

Thrombocytopenia – plts < 150

Symptoms when plts < 10

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7
Q

What are disorders of coagulation and are they more often inherited or acquired?

A

Due to a defect in the coagulation cascade

Inherited causes (rare)

Acquired causes (common)

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8
Q

What are the symptoms of thrombocytopaenia?

A

Epistaxis, GI bleeds, menorrhagia, bruising

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9
Q

What are the inherited and acquired causes of thrombocytopaenia?

A

Inherited causes (rare)

Acquired csuses (common)

eg: immune thrombocytopaenia (ITP), drug-related, DIC

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10
Q

What condition is this?

A

ITP = immune thrombocytopenia

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11
Q

For immune thrombocytopaenia, when do you treat and what the 4 treatment options?

A

Treatment options if bleeding or platelets < 20:

1) steroids and/or intravenous immunoglobulins
2) thromboietin agonists e.g., romiplostim
3) immunosuppression e.g., rituximab
4) splenectomy

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12
Q

In disorders of platelet function, is the platelet count low, normal or high?

A

Normal

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13
Q

What are the causes of disorders of platelet function?

A

Inherited:

Many rare diagnoses e.g., Glanzmann’s thrombasthenia

Acquired:

Drugs – much more common e.g., Aspirin, NSAIDs

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14
Q

Draw the coagulation cascade

A
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15
Q

What are the 3 main coagulation tests and which pathway do they test?

A

APTT = activated partial thromboplastin time

Assesses the intrinsic pathway

PT = prothrombin time

Assesses the extrinsic pathway

TT = thrombin time

Assesses terminal common pathway

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16
Q

Name 3 other coagulation tests which don’t test specific pathways in the coagulation cascade

A

Fibrinogen level

Clotting factor assays (normal level 100%)

D-dimers = breakdown products of fibrin clot

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17
Q

What are the 2 types of haemophilia and what is the deficiency?

A

Haemophilia A – deficiency of factor VIII

Haemophilia B – deficiency of factor IX (also known as Christmas disease)

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18
Q

What type of genetic inheritance is haemophilia A and B?

A

Both X-linked conditions affecting males only

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19
Q

What are the clinical features of haemophilia?

A

◦Spontaneous bleeding into joints and muscle

◦Unexpected post-operative bleeding

◦Chronic debilitating joint disease

◦Family history in majority of cases

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20
Q

What clinical features is this and what dieases does it occur in?

A

Major haematoma

Haemophilia

21
Q

What clinical feature is this and which disease does it occur in?

A

Haemarthrosis

22
Q

What clinical feature does this show and what disease does it occur in?

A

Chronic joint deformity

Haemophilia

23
Q

Which family member passes haemophilia to which family member?

A

Carrier mums have haemophiliac sons

24
Q

Draw a genetic diagram for a carrier mum and normal father (haemophilia)

A
25
Q

What happens when a father with haemophilia has children?

Draw a genetic diagram for this

A

Dads pass gene to carrier daughters

26
Q

What 3 tests are done to diagnose haemophilia and what do they test?

A

Prolonged APTT

tests factors VIII, IX, XI, XII in the intrinsic pathway

Normal PT

tests factors II, V, VII, X in the extrinsic pathway

Low factor VIII or IX levels

◦<1% = severe; 1-5% = moderate; >5% = mild haemophilia

27
Q

What is the treatment for haemophilia?

A
28
Q

What is the genetic inheritence pattern for von Villebrand disease?

A

Autosomal dominant

29
Q

What is von Villebrand disease?

A

vWD = deficiency of vWF and FVIII in plasma

von Willebrand factor = large multimeric protein that carries factor VIII in the blood

vWF also binds platelets to endothelial collagen

30
Q

What are the symptoms of von Villebrand disease?

A

Mucocutaneous bleeds, nosebleeds, menorrhagia

31
Q

What 6 test results would be abnormal in von Villebrand disease?

A

Prolonged APTT

Normal PT

Low vWF antigen level and/or vWF function

Low factor VIII level

Prolonged bleeding time

Defective platelet function

32
Q

What is the treatment for von Villebrand disease and how does it work?

A

Treatment with desmopressin (DDAVP), anti-fibrinolytics and plasma products

DDAVP stimulates release of vWF from Weibel-Palade storage bodies from endothelial cells

33
Q

What are the 3 acquired disorders of coagulation?

A

Liver disease

◦Deficient synthesis of clotting proteins

◦Impaired platelet function and fibrinolysis

Vitamin K deficiency

◦Infants who do not receive vitamin K at birth

◦Malabsorption due to jaundice

Disseminated intravascular coagulation (DIC)

◦Release of pro-coagulant material into circulation

◦Results in consumption of clotting factors

Causes both bleeding and thrombosis to occur

34
Q

What does this picture show?

A

Meningococcal DIC

Disseminated intravascular coagulation (DIC)

35
Q

What tests would be abnormal in disseminated intravascular coagulation (DIC)?

A
  • Prolonged PT, APTT, TT
  • Low fibrinogen, low platelets
  • Raised D-dimers or FDPs
36
Q

What disease is this?

A

Disseminated intravascular coagulation (DIC)

37
Q

Name 3 causes of DIC

A

cancer, sepsis, obstetric disasters

38
Q

What does iatrogenic mean?

A

Overdose of anti-coagulant drugs

39
Q

Name the 3 types of anti-coagulants

A

Heparin – used to treat MIs, PEs, DVTs

Warfarin – used to treat PEs, DVTs, AF, prosthetic valves

DOACs = direct oral anti-coagulants

  • Direct thombin inhibitors (dabigatran, argatroban)
  • Factor Xa inhibitors (rivaroxaban, apixaban)
40
Q

What medication caused this?

A
41
Q

What is the role of vitamin K in clotting?

A

Vitamin K is required for gamma-carboxylation of factors II, VII, IX, X

42
Q

What drug inhibits vitamin K?

A

Inhibited by warfarin (monitored using the INR)

43
Q

What are the 3 causes of deficiency of vitamin K?

A

◦Malabsorption of vitamin K

◦Biliary obstruction (jaundice)

◦Haemorrhagic disease of the newborn (give 1mg at birth)

44
Q

What is the extrinsic pathway?

A

factors II,V,VII,X,

monitored by PT (basis of the INR)

45
Q

What is the intrinsic pathway?

A

factors VIII, IX, (also XI and XII)

monitored by APTT (APTR = APTT ratio)

46
Q

What do both intrinsic and extrinsic pathways activate?

A

Both pathways activate prothrombinase

(factors V, X, Ca and phospholipid)

47
Q

What happens when prothrombin is activated?

A

}Prothrombin is activated leading to a “thrombin burst”

48
Q

What does the cleavage of fibrinogen form?

A

Fibrin and clot formation

103 Theme 2 - Blood (10 decks)

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