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Y5 Haematology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

How do coagulation disorders present?

A

Bleeding into deep tissues, muscles, joints; delayed but severe bleeding after injury (often prolonged)

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2
Q

How do vascular defects present?

A

Superficial bleeding into skin/mucosal membranes = petechia, purpura, easy bruising ; bleeding immediately after injury

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3
Q

How do platelet disorders present?

A

Superficial bleeding into skin/mucosal membranes; bleeding immediately after injury

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4
Q

Name some congenital causes of vascular defects?

A

Osler-Weber-Rendu Syndrome, Ehlers-Danlos Syndrome, Marfans, Pseudoxanthoma elasticum, Osteogenesis Imperfectaother CTDs

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5
Q

What are the main causes of acquired vascular defects?

A

Senile purpura, infection, steroids, scurvy

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6
Q

Name 3 infections that commonly present with vascular defects

A

Measles, Dengue fever, Meningococcal infections

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7
Q

What vascular presentation is seen in scurvy?

A

Perifollicular haemorrhages

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8
Q

What is another name for Osler-Weber-Rendu Syndrome?

A

Hereditary Haemmorhagic Telengiectasia

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9
Q

Bleeding disorders are split into 4 groups, based on their aetiology. List these groups.

A

Vascular defects (easy bruising), Platelet disorders (low or abnormal function), coagulation disorders (factor deficiency), mixed

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10
Q

What is the inheritance pattern of Osler-Weber-Rendu Syndrome? What 2 genes are commonly affected?

A

Autosomal Dominant; ENG, ALK1

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11
Q

What is the diagnostic criteria for OWR Syndrome?

A

Clinical diagsnosis. 3/4: epistaxis, telengiectasia, visceral lesions, FHx

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12
Q

What happen in OWR syndrome/HHT?

A

Vascular dysplasia (mucocutaneous telengiectasia, arteriovenous malformation) causes increased bleeding

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13
Q

What is the definition of Thrombocytopenia?

A

Platele count under 150 bill g/L

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14
Q

What are the acquired casues of decreased platelet function?

A

Aspirin, Cardiopulmonary bypass, Uraemia

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15
Q

What are the congenital causes of decreased platelet function?

A

Storage pool disease, Thrombasthenia (glycopprotein deficiency)

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16
Q

Causes of thrombocytopenia?

A

Low Production: bone marrow failure

Increased destruction: AITP, ITP, Drugs (eg Heparin), DIC, HUS, TTP

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17
Q

Epidemiology of Acute ITP

A

Children (2-6y), F:M 1:1

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18
Q

Aetiology of Acute ITP

A

Most commonly preceded by an infection

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19
Q

How quickly does Acute ITP present and how long does it last?

A

Abrupt onset, lasts 2-6 weeks

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20
Q

Treatment of Acute ITP?

A

Normally self limiting - spontaneous remission

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21
Q

Epidemiology of Chronic ITP

A

Adults; F:M 3:1

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22
Q

How quickly does Chronic ITP present and how long does it last?

A

Symptoms can present abruptly or indolently; symptoms are normally long term

23
Q

Aetiology of Chronic ITP?

A

Associated with Autoimmune Disease, CLL and HIV

24
Q

Treatment of Chronic ITP

A

IVIg, steroids, splenectomy

25
Q

What are the 3 main inherited coagulation disorders?

A

Haemophilia A, Haemophilia B, von Willebrand’s disease

26
Q

What are the 3 main acquired causes Coagulation disorders?

A

DIC, Liver Disease, Vit K deficiency

27
Q

What factor is deficient in Haemophilia A, Haemophilia B, vW Disease?

A

Haemophilia A = Factor VIII, Haemophilia B = Factor IX, von Willebrand’s disease = Factor VIII (also decreased platelet function)

28
Q

What is another name for Haemophilia B?

A

Christmas Disease

29
Q

Inheritance pattern of Haemophilia A?

A

X linked recessive

30
Q

Inheritance pattern of Haemophilia B?

A

X linked recessive

31
Q

Is Haemophilia A or B more common?

A

A = 1/10000 males (B=1/50000)

32
Q

How is vW Disease most commonly inherited?

A

Autosomal Dominant

33
Q

Mgmt for Haemophilia A

A

Avoid NSAIDs and IM injections, desmopressin, lifelong factor VIII rconcentrates

34
Q

Mgmt for Haemophilia B

A

Factor IX concentrates

35
Q

Mgmt for VW Disease

A

Desmopressin, VWF and Factor VIII concentrates

36
Q

What factors require Vit K for synthesis?

A

2, 7, 9, 10

37
Q

Which factors have impaired synthesis in Liver disease?

What else is affected?

A

Decreased synthesis of Factors of 2, 7, 9, 10, 11, and fibrinogen
Decreased absorption of Vit K
Abnormalities of platelet function

38
Q

Treatment for Vit K deficiency

A

IV vitamin K or FFP for acute haemorrhage

39
Q

What is the platelet count at presentation for Acute ITP and Chronic ITP?

A

Acute ITP = under 20,000 Chronic ITP = under 50,000

40
Q

How is Haemophilia graded? Define each grade.

A

Related to factor level: Severe = under 1%, moderate = 1-5%; mild = 5-25%

41
Q

How does Haemophilia (A&B) present clinically?

A

Often present early in life or with prolonged bleeding after surgery/trauma

42
Q

How does vW disease present clincally?

A

often bleeding indicative of platelet disroders (i.e mucocutaneous bleeding) but can also include bleeding indicative of coagulation disorders

43
Q

What are the causes of Vitamin K Deficiency?

A

Vitamin K malabsorption/malnutrition, Warfarin, Abx therapy, biliary obstruction

44
Q

What are the causes of DIC?

A

Malignancy, sepsis, trauma, obstetric complication, toxins

45
Q

Treatment for DIC

A

Treat the cause, give transfusions, FFP, platelets, cryo etc.

46
Q

What happens in DIC?

A

Widespread activation of coagulation causes clotting factors and platelets to be consumed which then causes increased risk of bleeding

47
Q

Clotting Results for Heparin: INR, APTT, TT, Platelet Count, Bleeding Time

A 
INR - high
APTT - very high 
TT - very high
Platelet Count - normal
Bleeding Time - normal
48
Q

Clotting Results for DIC: INR, APTT, TT, Platelet Count, Bleeding Time

A 
INR - very high
APTT - very high
TT - very high
Platelet Count - low
Bleeding Time - high
Also high D-dimer
49
Q

Clotting Results for Liver Disease: INR, APTT, TT, Platelet Count, Bleeding Time

A 
INR - high
APTT - high
TT - normal or high
Platelet Count - normal or down
Bleeding Time - normal or high
Also high AST
50
Q

Clotting Results for Platelet Defect: INR, APTT, TT, Platelet Count, Bleeding Time

A 
INR - normal
APTT - normal
TT - normal
Platelet Count - normal 
Bleeding Time - increase
51
Q

Clotting Results for Vit K Deficiency: INR, APTT, TT, Platelet Count, Bleeding Time

A 
INR - very high 
APTT - high
TT - normal
Platelet Count - normal 
Bleeding Time - normal
52
Q

Clotting Results for Haemophilia: INR, APTT, TT, Platelet Count, Bleeding Time

A 
INR - normal 
APTT - very high
TT - normal
Platelet Count - normal 
Bleeding Time - normal
53
Q

Clotting Results for Von Willebrand’s Disease: INR, APTT, TT, Platelet Count, Bleeding Time

A 
INR - normal
APTT - very high
TT - normal 
Platelet Count - normal 
Bleeding Time - high

Y5 Haematology (10 decks)

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