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Haematology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

List 3 causes of vascular abnormalities associated with primary Haemostasis failure

A

Vit C deficiency (scurvy) (reduced collagen synthesis),
Vasculitis (henoch-schonlein purpura),
Steroids (reduces vessel wall collagen)

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2
Q

What is a purpura rash

A

Red or purple spots/patches/bruises
On skin or mucosal membrane
Are non-blanching
Are caused by broken blood vessels/ bleeding

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3
Q

List 4 causes of acquired thrombocytopenia (leading to primary haemostatic failure)

A
  • Malignancy in the bone marrow (reduced production)
  • Coagulopathy e.g. DIC (increased destruction)
  • Autoimmune e.g. ITP (increased destruction)
  • hypersplenism (increased destruction)
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4
Q

List 2 drugs that can cause defects in platelet function

A
  • Aspirin
  • NSAIDs e.g. ibuprofen
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5
Q

Failure of what organ can lead to defect in platelet function

A

Renal failure

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6
Q

Is vWF deficiency usually acquired or hereditary? If hereditary, what inheritance does it display?

A

Hereditary, autosomal dominant

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7
Q

What is the most common cause of primary haemostatic failure and how would this direct your investigations & treatment

A

Thrombocytopenia
- If pancytopenia - biopsy to check for marrow malignancy
- If only thrombocytopenia - assume ITP (unless specific signs of other cause) and start immunosuppression

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8
Q

Name 3 causes of multiple factor deficiency associated with secondary haemostatic failure

A
  • Liver failure (reduced synthesis)
  • Vitamin K deficiency/ warfarin therapy (reduced ‘activation’)
  • Complex coagulopathy (DIC) (increased use)
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9
Q

What are the two screening tests for secondary haemostasis and what factors/pathways do they assess

A

Prothrombin Time (PT)
- Extrinsic (TF/VII) & common pathway

Activated partial thromboplastin time (APTT)
- Intrinsic (VIII/IX) & common pathway

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10
Q

What is a screening test for primary haemostatic failure

A

Platelet count

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11
Q

Where are coagulation factors synthesised

A

Hepatocytes

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12
Q

Which 4 factors are carboxylated by vitamin K in order to function

A

II (prothrombin), VII, IX, X

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13
Q

What are the two sources of vitamin K

A

Intestinal absorption from diet (jejunum & ileum)
Intestinal synthesis (large intestine)

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14
Q

What is required for vitamin K absorption

A

Bile salts

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15
Q

List 4 causes of vitamin K deficiency

A
  • Poor dietary intake
  • Malabsorption conditions
  • Obstructive jaundice (bile duct obstructed)
  • Vitamin K antagonists e.g. warfarin

(+ haemorrhagic disease of newborn)

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16
Q

Why are all baby’s given vitamin K at birth

A

Newborns have very low vitamin K as it can’t easily cross teh placenta and is not in breastmilk

Vitamin K deficiency in newborns can cause haemorrhagic disease of the newborn

Therefore, newborns must be injected with vitamin K at birth

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17
Q

What is disseminated intravascular coagulation, describe its pathophysiology

A

1) Cytokine signalling as part of inflammatory response OR
Release of procoagulant material e.g. TF

2) Excessive activation of haemostatic system
(Primary, secondary & thrombolysis)

3) Microvascular thrombus formation => end organ damage

4) Clotting factor consumption & hence depletion => brusing, purpura, generalised bleeding

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18
Q

What test can be used to differentiate between DIC and other causes of multiple factor deficiencies

A

D-dimer test (d-dimer is a fibrin degradation product)

DIC causes excessive fibrinolysis, unlike in other causes of multiple factor deficiency

19
Q

DIC aetiology

A
  • Sepsis
  • Obstetric emergencies
  • Malignancy
  • Hypovolaemic shock
  • ABO incompatible blood transfusion
20
Q

DIC clinical presentation

A
  • Petechiae/purpura/bruising
  • Excessive bleeding
  • Acutely ill - fevers, confusion, hypotension, potential coma
21
Q

DIC investigations & findings summary

A
  • Severe thrombocytopenia (low platelet count)
  • Prolonged PT, APTT & TT (low clotting factors)
  • High FDPs e.g. d-dimer (excess fibrinolysis)
  • Fragmented RBCs on blood film
22
Q

DIC treatment

A

Treat cause!
Replacement therapy!
- Platelet transfusions
- Plasma transfusions
- Fibrinogen replacement

23
Q

What is haemophilia and what is its inheritance pattern

A

X-linked, hereditary disorder (more likely in men)
Abnormal, prolonged, episodic bleeding at one or a few sites

24
Q

Haemophilia A vs B, what factor does it affect?

A

Haemophilia A - Factor VIII deficiency (most common)
Haemophilia B - Factor IX deficiency

25
Q

How is primary haemostasis affected with haemophilia

A

Primary haemostasis is normal

26
Q

Severe haemophilia clinical presentation

A

Recurrent haemarthroses (knee, ankle, elbow)
Recurrent soft tissue bleed (extensive bruising in kids)
Prolonged bleeding after medical/ dental procedures

27
Q

Thinking about the factors affected in haemophilia, what screening tests would likely be abnormal?

A

Haemophilia A & B affects the intrinsic pathway
& so APTT would be prolonged
& PT would be normal

28
Q

Haemophilia investigations summary

A

Bloods - APTT (prolonged), PT (normal)
Bloods - coagulation factor assays (VII - A, IX - B)
Genetic testing

29
Q

Haemophilia long term management & risk

A

Prophylactic clotting factor replacement
- in severe disease only
- risk of individual developing antibodies against the factor

30
Q

Summarise primary haemostatic failure aetiology

A

Vascular (collagen) defect (usually acquired)
- Severe vitamin C/ scurvy
- Steroids
- Vasculitis e.g. henoch-schonlein purpura

Thrombocytopenia (low platelets) (usually acquired)
- Reduced production - marrow malignancy
- Increased destruction - DIC, ITP, splenomegaly

vWF deficiency (usually herediatry)

31
Q

Summarise secondary haemostatic failure aetiology

A

Multiple factor deficiency (usually acquired)
- Liver disease (reduced production)
- DIC (increased use)
- Vitamin K deficiency (reduced ‘activation’)
- malnutrition/absorption/obstructive jaundice/warfarin

Single factor deficiency (usually hereditary)
- Haemophilia A or B

32
Q

If a patient with haemophilia (mild or severe disease) was acutely bleeding or was about to undergo surgery, would treatment would you provide

A
  • Minor bleed - Desmopressin (stimulates vWF which stimulates clotting factor)
  • Major bleed - Clotting factor (VIII or IX) infusion
  • Bleeding wounds (not muscle haematoma/haemarthrosis/haematuria) - Option of anti-fibrinolytics
33
Q

What screening tests would be affected in a multiple factor deficiency such as liver disease/DIC/vitamin K deficiency

A

All! - PTT, APTT & TT

34
Q

What does an isolated prolonged APTT mean?

A

Haemophilia A or B

35
Q

What is Von willebrand’s disease, what effect does it have on primary haemostasis & factor VIII

A
  • Inherited bleeding disorder
  • Reduced quantity or function of vWF, varying severity
  • low vWF => impaired platelet adhesion to subendothelium
  • low vWF => factor VIII deficiency (as vWF ‘protects’ VIII)
36
Q

vWF investigations & findings

A

FBC
- normal
- or low platelet count
- or microcytic anaemia (iron deficiency)

Coagulation tests
- PT normal
- APTT normal or prolonged if factor VIII deficiency

Specialised vWF tests

37
Q

What is the relationship between vWF and factor VIII

A

VWF protects factor VIII from proteolysis

38
Q

VWF treatment

A

Desmopressin OR
Plasma-derived factor VIII concentrates with intact vWF

39
Q

What reaction is involved in immune thrombocytopenia purpura (ITP)

A

Type II hypersensitivity - Antibody production against platelets

40
Q

Immune thrombocytopenia purpura (ITP) an isolated (primary) conditions or can be secondary to another disorder. Name 5 disorders associated with ITP

A
  • Autoimmune - SLE, APS
  • Infections - viral, helicobacter pylori
  • Medications - antibiotics, anticonvulsants, NSAIDs…
  • Lymphoproliferative disorders

**Viral infection in children most common!!

41
Q

Immune thrombocytopenia purpura (ITP) clinical presentation

A
  • History of recent viral illness
  • Usually children <10yrs
  • 1 or 2 day history of bleeding/ bruising
    • bleeding gums, epistaxis, menorrhagia
    • petechia or purpura rash
42
Q

Immune thrombocytopenia purpura (ITP) investigations & findings

A

FBC & blood film - isolated thrombocytopenia

43
Q

Immune thrombocytopenia purpura (ITP) management

A

Usually self-limiting, monitoring till normal platelets
If severe or actively bleeding
- prednisolone => IV immunoglobulins => blood transfusion

Haematology (22 decks)

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