Coagulation disorders

Question 1

What are the two types of haemophilia?

Answer 1

Haemophilia A and B
Both X linked
A: decreased factor 8
B: decreased factor 9

Question 2

How would you treat haemophilia?

Answer 2

replace missing clotting factors during bleed - avoid NSAIDs and IM injections

Question 3

What is Von Willebrand’s disease?

Answer 3

prevents factor 8 destruction
autosomal dominant
causes bleeds and easy bruising

Question 4

How do you treat Von Willebrand’s disease?

Answer 4

treat a large bleed - vasopressin and vWF concentrate

Question 5

What is DIC?

Answer 5

disseminated intravascular coagulation - formation of microvascular thrombi and multi-organ failure = consumption of fibrinogen, platelets and clotting factors = haemorrhage

Question 6

What can cause DIC?

Answer 6

sepsis, cancer, trauma, anaphylaxis

Question 7

What is thrombophilia?

Answer 7

triad of statis, hypercoagulability, vessel wall injury
which can cause DVT
Test for it with D-dimer

Question 8

What is ITP?

Answer 8

immune thrombocytopenia
- autoimmune low platelet count
causes purpuric rash and bleeding

Question 9

How would haemophilia cause chronic joint deformity?

Answer 9

By spontaneous bleeds into the joint causing haemotoma and deformity

Question 10

How could someone get haemophilia A later in life?

Answer 10

acquired later in life through autoantibodies against factor VIII

Question 11

What would INR and APTT tests show in haemophilia A?

Answer 11

INR normal

APTT prolonged

Question 12

What should haemophiliacs avoid?

Answer 12

NSAIDs, IM injections, contact sports

Question 13

How would you treat haemophilia A?

Answer 13

purified factor VIII
factor VIII in fresh frozen plasma
desmopressin (analgue of vasopressin)

Question 14

How would someone acutely present with haemophilia and what is the pathophysiology?

Answer 14

compartment syndrome
- swollen, tight, pale shiny,
- swollen muscle compresses blood vessels and nerve
treat with fasciotomy

Question 15

What are three common causes of raised platelets?

Answer 15

inflammation
malignancy
bleeding

Question 16

What is classic test result that indicates haemophilia?

Answer 16

prolonged APPT

Question 17

What would tests for DIC show?

Answer 17

low - platelets, fibrinogen

high - prothrombin time, d-dimer

Question 18

What are some conditions that cause excessive clotting?

Answer 18

DIC (widespread activation of clotting cascade)

TTP (deficiency of protease and excessive VWF)

Question 19

What coagulation factors are vitamin K dependant?

Answer 19

2, 7, 9. 10

Question 20

What are some problems that cause bleeding?

Answer 20

over-anticoagulation
DIC
ITP

Question 21

What is HUS?

Answer 21

haemolytic uremic syndrome =

microangiopathic haemolytic anaemia + thrombocytopenia + AKI

Question 22

What is TTP?

Answer 22

thrombotic thrombocytopenic purpura =

HUS + neurological abnormalities + fever

Question 23

Which test would be prolonged in antiphospholipid syndrome?

Answer 23

APTT

Question 24

What are some congenital thombophilias?

Answer 24

protein C & S deficiency
Factor V leiden
Antithrombin III

Question 25

What is in the extrinsic pathway? How is it measured?

Answer 25

TF - VII

protrombin time/INR

Question 26

What are in the instrinsic pathway and how is it measured?

Answer 26

XII - XI - IX - VIII

APTT

Question 27

What is in the common pathway and how is it measured?

Answer 27

X - II - I

APTT, PT