Bleeding Disorders Flashcards Preview

Yr3 Haematology > Bleeding Disorders > Flashcards

Flashcards in Bleeding Disorders Deck (18)
Loading flashcards...
1

What in a history would suggest a bleeding disorder rather than a normal bleed?

Large post-surgical bleeding or menorrhagia, frequent nosebleeds or bruising etc

Innapropriate bleeding e.g. after a vaccination or unprovoked

2

The pattern of bleed is important as it indicates what kind of disorder you may have. How would a platelet deficiency bleed?

Mucosal bleeds
Epistaxis
Purpura & petechiae
Menorrhagia
GI bleeds

3

What kind of bleeds would happen in someone with a coagulation factor problem?

Articular bleeds
muscle Haematoma
CNS bleeds

4

How would you investigate a patient's history to determine if its a congenital or acquired bleeding disorder?

FH
Previous episodes
Age at first event
Previous surgical challenges
Associated History

5

What is the most common heritable coagulation factor disorder?

Haemophilia A & B
A = Factor 8
B = Factor 9

Lumped together because the phenotype is identical

6

What determines severity of Haemophilia?

Residual Coagulation Factor Activity
Severe = <1%
Mod = <5%
Mild = <30%

7

How does a person with haemophilia present?

Obviously with loads of bleeds (spontaneously, excessively or on little stimulation)
Bleeds will be:
- Haemarthrosis
- Muscle Haematoma
- CNS bleeds
- Peritoneal Bleeds
- Post-surgical bleeds

8

Other than obviously bleeding loads, what's the big issue with haemophilia?

Patients tend to bleed into hinge, weight bearing joints i.e. knee
This leads to Chronic Haemophilic Arthropathy, with most patients developing end stage joint disease by 30 if they're not treated properly

9

How do we go about diagnosing a haemophilia if we suspect it from history?

FBC + aPTT + PT
Coagulation Facotr 8/9 Assay
Genetic Tests

10

How can we manage acute bleeding events in haemophilia?

Coagulation Factor replacement
Desmopressin
Tranexamic Acid

11

What can we give patients as prophylaxis for haemophilia?

Regular Coagulation Factor Injs
Splints & Physio
Analgesia
Synovectomy
Joint Replacement

12

Treating Haemophilia comes with it's own complications, what the majorones?

1) Viral infections e.g. HIV from donated coagulation factors (rare today as we use recombinant ones)

2) Development of Anti-factor Abs

3) Desmopressin SEs

13

How do haemophilia patients develop Anti-factor Abs?

Patients with a null mutation (meaning they completely lack the Factor, mostly in Factor 8 aka haemophilia A) i.e. Severe Haemophilia

When they're eventually exposed to the Factor it's foreign to them so their bodies produce Anti-Factor8 Antibodies

14

What are the complications of Desmopressin?

Can cause MI so contraindicated in IHD

Can cause Hyponatraemia in infants --> Seizures so contraindicated under 3 yrs

15

What is the most common platelet disorder?

von Willebrand's Disease

1 in 200, making it the most common heritable bleeding disorder by far

16

How are haemophlia & von Willebrand's Disease inherited?

Haemophilia = X-linked

von Willebrand's = Autosomal

17

What are the types of von Willebrand's?

1 = Quantitative deficiency
2 = Qualitative deficiency
3 = Complete deficiency

18

How do we manage von Willebrand's?

vWF concentrate or Desmopressin

Tranexamic Acid

Topical Applications

OCP