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Haematology > Bleeding Disorders > Flashcards

Bleeding Disorders Flashcards

1
Q

What is important to ask in history of bleeding disorder

A 
HPC 
Easy bruising / how long to disappear 
Epistaxis - normal = <15 minutes 
Post surgical - dental / tonsils / vaccinations 
Menorrhagia
PPH
Post-trauma
Any joint pain / limp

PMH
Drug history
Symptoms of anaemia
Systemic Sx - B symptoms / recent infection
FH of bleeding disorder / heavy bleeding / menorrhagia
FH of autoimmune

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2
Q

What is platelet type bleeding / vWF

A 
Mucosal
Epistaxis
Purpura
Petechiae 
Menorrhagia
GI bleeding
Retinal haemorrhage 
Ecchymosis
Easily bruising
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3
Q

What is coagulation factor type bleeding

A

Bleeding into articular joints
Unprovoked muscle haematoma
Brain haemorrhage

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4
Q

How does muscle haematoma present

A

Swollen hot leg

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5
Q

What is important in Hx to determine if congenital or acquired / type of inheritance

A 
Previous episodes
Age of first event
Previous challenges with surgery
FH 
Sex
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6
Q

What is haemorrhagic diathesis

A

Unusualy susceptibility to bleed

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7
Q

What causes

A

Inhibtion of function of
Platelets
vWF
Coagulation factor

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8
Q

What is haemophilia

A

X-linked disorder causing coagulation factor deficiency
A = factor 8 (VII) deficiency = most common
B = factor 9 (IX)

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9
Q

What are the clinical features of haemophilia

A 
Risk of severe bleeding in response to minor trauma or spontaneous 
Haemarthrosis - bleed into joint 
Muscle haematoma - calf + biceps 
CNS bleeding
Retroperitoneal bleeding
Post surgical bleeding
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10
Q

What are the complications of haemophilia

A 
Synovitis 
Destruction of cartilage
Chronic haemophilic arthropathy
Neurovascular compression + compartment syndrome 
Haemorrhagic stroke
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11
Q

How and when does haemophilia present

A 
6 months - 2 years when starting to walk 
Fall over 
Stop walking as bleed into knee
Bruising 
Swollen painful leg
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12
Q

How do you Dx haemophilia

A

Reduced factor 8 or 9
Prolonged APTT
Normal PT
Genetic test but unlikely to have FH

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13
Q

How do you treat haemophilia bleeding

A

Coagulation factor replacement
DON’t transfuse FFP unless no concentrate factor
DDAVP - releases stored factor 8 + VWf

Tranexamic acid if minor bleed
Recombinant factor if major bleed
Gene therapy

Prophylaxis if severe

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14
Q

When do you give prophylaxis factor concentrate

A

Severe haemophilia to keep level of factor >2% so don’t bleed spontaneously
Contine for 45 weeks of the year

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15
Q

How do you manage haemophilia

A 
Splints
Physio
ANalgesia
Synovectomy
Joint replacement 
Give vaccines SC instead of IM
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16
Q

What are the complications of treatment

A

Viral infection - HIV, HBV, HCV, CJD

Development of inhibitors to factor

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17
Q

What are the CI of DDAVP

A

Elderly as stroke / MI risk

<3 as hyponatraemia / fits

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18
Q

What does severity depend on

A

Amount of residual coagulation factor in the circulation

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19
Q

What is severe

A

<1%

Will bleeding spontaneously into muscles and joints every few weeks

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20
Q

What is moderate

A

1-5%

DONT bleed spontaneous

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21
Q

What is mild

A

5-30%

Don’t come to harm but may need procedures in place for surgical procedures

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22
Q

What is vWF

A

AD defect in vWF leading to platelet type bleeding (factor VIII in clotting cascade)

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23
Q

What are the types of vWF

A

Type 1 = quantitative - don’t make enough
Type 2 = qualitative - abnormal function
Type 3 = severe complete deficiency RARE

24
Q

How do you Dx and Rx and what should you check

What vaccine

A

Dx = low levels of vWF in blood
Check blood group as O blood has low levels of vWF

Rx
Tranexamic acid if mild e.g. menorrhagia
DDAVP raises level of vWF by stimulating release = 1st line
vWF concentrate / factor VII for minority if severe

Immunisation against hep A and B
Iron if deficient

25
Q

What are acquired causes of bleeding

A

Thrombocytopenia
Liver and renal failure
DIC
Anti-coagulants + platelets

26
Q

What causes severe thrombocytopenia due to increased destruction

A

ITP
DIC
TTP
Malignancy

27
Q

What leads to decreased production of platelet

A 
Marrow failure - leukaemia / myeloma / myelodisplastic
Sepsis 
B12 / folic acid deficiency 
Liver failure 
Cytotoxic drugs / RT 
Aplastic anaemia 
SLE
Anti-phospholipd syndrome
28
Q

What causes increased consumption

A 
ITP / TTP / heparin induced
DIC
HUS 
Viral infection - EBV / HIV / hepatitis 
Pregnancy
B12 deficiency
Alcohol
Drugs
Hypersplenism
29
Q

How does hypersplenism cause low platelets

A

Platelet sticks to big spleen

30
Q

What is ITP

A

Immune mediated reduction in platelet count
Type 2 hypersensitivity where Ab destroy platelet
Isolated blood thrombocytopenia

31
Q

What is associated with ITP

A 
Common after viral infection / vaccinations 
Collagenosis
Lymphoma / CLL 
Drug induced
Can be idiopathic
32
Q

What type of ITP do you get

A

Acute

Chronic relapsing remitting

33
Q

Who tends to get acute

A

More common in children following infection or vaccination

Usually self limiting

34
Q

How does it present and what are complications

A

Isolated blood thrombocytopenia
Platelet type bleeding - petehiae/ bruising
Well in themselves
Normal APTT

Chronic ITP
Anaemia
Haemorrhage / GI Bleed

35
Q

How do you Dx

A 
History 
PROCESS OF EXCLUSION 
FBC - thrombocytopenia
Do blood film to make sure its okay / exclude ALL (tests at bottom) 
Must exclude heparin induced 
Rarely look at marrow
36
Q

How do you Rx

A

Usually self limiting
None if no active bleeding
Advise against contact sport / IM injection / anti-coagulant / NSAID
Do repeat bloods to check resolved

If bleeding / severe thrombocytopenia <10
Steroids + PPI
IV IgG
May need transfusion but doesn’t work as Ab still destroy - only give if severe bleeding

Other if unresponsive 
Splenectomy
Ritixumab - 
Thrombopoetin analogue
Azahthioprine - 1st line in pregnancy
37
Q

What is Evan’s syndrome

A

ITP + autoimmune haemolytic anaemia

38
Q

How does liver cause bleeding disorder

A

Liver produces clotting factors and fibrinogen

39
Q

What are lab results in liver failure

A

Prolonged PT + APTT
Reduced fibirnogen
Abnormal LFT

40
Q

Where do most bleeding come from

A

Structural lesions e.g. varices

May have other features of liver failure

41
Q

What is associated

A

CHolestasis

Vit K dependent factor deficiency

42
Q

How do you Rx

A

Replace FFP

Give vit K

43
Q

What causes haemorrhagic disease of the new born

A

Immature coagulation system

Vit K deficient diet

44
Q

What does it lead too

A

Fatal haemorrhage

45
Q

How do you Rx

A

Give IM vit K at birth

46
Q

What causes acquire haemophilia

A

Autoimmune
Elderly
Malignancy
Pregnancy

47
Q

How do you Dx and Rx

A

Raised APTT

Steroids

48
Q

CI to platelet

A

Chronic bone marrow failure
TTP
Autoimmune thrombocytopenia

49
Q

What is heparin induced thrombocytopenia

A

Development of Ab to platelet due to exposure to heparin
Usually 5-10 days after
Causes a hyper coagulable state and can cause thrombosis even though low platelet
Also break down platelet = thrombycotpenia
So you get a patient on heparin with low platelet forming blood clots

50
Q

How do you Dx

A

HIT Ab

51
Q

How do you Rx

A

Stop heparin

52
Q

What are other causes of drug induced thrombocytopenia

A 
Quinine 
Abiximab 
NSAID
Diuretic - furosemide
Ax - penicillin, sulphonamide, rifampicin 
AED - carbamazepine + valproate
53
Q

What should you do for petechiae / non-blanching rash

A

Urgent FBC if no definite cause known e.g. viral infection / changes to medication / PMH

54
Q

Does normal WBC rule out infection

A

No because infection stimulates recruitment but also uses up WBC so can be normal

55
Q

What investigations after blood shows thrombocytopenia

A

Blood film - malignancy / b12 / folate / haemolytic
Reticulocyte count
- If high = haemolytis / bleed
- If low = marrow failure
U+E - HUS / TTP / DIC can affect renal
Clotting screen - synthetic function of liver and DIC
B12 / folate - can cause cytopenia’s

56
Q

How could pancreatic mass cause prolonged PT

A

Vit K deficeincy due to biliary obstruction

Haematology (13 decks)

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