Coagulation disorders Flashcards
What are the two types of haemophilia?
Haemophilia A and B
Both X linked
A: decreased factor 8
B: decreased factor 9
How would you treat haemophilia?
replace missing clotting factors during bleed - avoid NSAIDs and IM injections
What is Von Willebrand’s disease?
prevents factor 8 destruction
autosomal dominant
causes bleeds and easy bruising
How do you treat Von Willebrand’s disease?
treat a large bleed - vasopressin and vWF concentrate
What is DIC?
disseminated intravascular coagulation - formation of microvascular thrombi and multi-organ failure = consumption of fibrinogen, platelets and clotting factors = haemorrhage
What can cause DIC?
sepsis, cancer, trauma, anaphylaxis
What is thrombophilia?
triad of statis, hypercoagulability, vessel wall injury
which can cause DVT
Test for it with D-dimer
What is ITP?
immune thrombocytopenia
- autoimmune low platelet count
causes purpuric rash and bleeding
How would haemophilia cause chronic joint deformity?
By spontaneous bleeds into the joint causing haemotoma and deformity
How could someone get haemophilia A later in life?
acquired later in life through autoantibodies against factor VIII
What would INR and APTT tests show in haemophilia A?
INR normal
APTT prolonged
What should haemophiliacs avoid?
NSAIDs, IM injections, contact sports
How would you treat haemophilia A?
purified factor VIII
factor VIII in fresh frozen plasma
desmopressin (analgue of vasopressin)
How would someone acutely present with haemophilia and what is the pathophysiology?
compartment syndrome
- swollen, tight, pale shiny,
- swollen muscle compresses blood vessels and nerve
treat with fasciotomy
What are three common causes of raised platelets?
inflammation
malignancy
bleeding
What is classic test result that indicates haemophilia?
prolonged APPT
What would tests for DIC show?
low - platelets, fibrinogen
high - prothrombin time, d-dimer
What are some conditions that cause excessive clotting?
DIC (widespread activation of clotting cascade)
TTP (deficiency of protease and excessive VWF)
What coagulation factors are vitamin K dependant?
2, 7, 9. 10
What are some problems that cause bleeding?
over-anticoagulation
DIC
ITP
What is HUS?
haemolytic uremic syndrome =
microangiopathic haemolytic anaemia + thrombocytopenia + AKI
What is TTP?
thrombotic thrombocytopenic purpura =
HUS + neurological abnormalities + fever
Which test would be prolonged in antiphospholipid syndrome?
APTT
What are some congenital thombophilias?
protein C & S deficiency
Factor V leiden
Antithrombin III
What is in the extrinsic pathway? How is it measured?
TF - VII
protrombin time/INR
What are in the instrinsic pathway and how is it measured?
XII - XI - IX - VIII
APTT
What is in the common pathway and how is it measured?
X - II - I
APTT, PT
