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Inherited and Acquired Coagulation Disorders

Terms in this set (16)

Haemophilia A (pathophysiology, inheritance, presentation, diagnosis)
8 deficiency
X-linked recessive
presents early in life or as prolonged bleed after surgery/trauma
diagnosed as increased APTT, normal PT, reduced F8 assay
treatment of Haemophilia A (4)
avoid NSAIDs
avoid IM injections
desmopressin (increases vWF release - a protector of 8)
f8 concentrates - life-long
haemophilia disease (pathophysiology, inheritance, management)
f9 deficiency
x-linked recessive
manage with f9 concentrates
vW disease types, which blood factors are affected, inheritance, presentation
many types:
- 1 is reduced number
- 2 (many types) is reduced function
- 3 is absent vWF
reduced vWF and F8 (as vWF protects f8 in circulation)
AD inheritance
presents as plt disorder bleeding, (or coagulation factor bleed)
diagnosis of vWD (6)
increased APTT, increased bleed time, decreased f8, decreased vWF, normal INR and plts
managment of vWD (3)
desmopressin
vWF concentrate
f8 concentrate
what is DIC, what are some causes (5), what are the blood differential results, how is it treated
widespread activation of coagulation resulting in factor and plt consumption so increased risk of thrombus and bleed
causes:
- malignancy
- sepsis
- trauma
- obstetric complications
- toxins
differentials:
- low platelets
- low fibrinogen
- high FDP/D-dimer
- long pt and inr
treatment
- transfusion
- ffp
- plt
- cryoprecipitates...
what does liver disease result in (factor synthesis, plt function, vK levels) (3)
decreased II, V, VII, IX, X, XI
decreased absorption of vK
abnormal plt funciton
vK deficiency - what factors are reliant, what are causes (4), what is treatment (2)
II, VII, IX, X; and C, S - explains why warfarin is procoagulant initially
causes:
- warfarin
- vK malabsorption/malnutrition
- Abx therapy
- Biliary obstruction
treatment
- IV vK or FFP
Heparin: INR, APTT, Thrombin time, Plt count, bleed time
inr high
aptt very high
thrombin time very high
plt normal
bleed time normal
DIC: INR, APTT, Thrombin time, Plt count, bleed time, d-dimer
inr very high
aptt very high
thrombin tim very high
plt count low
bleed time high
d-dimer high
liver disease: INR, APTT, Thrombin time, Plt count, bleed time, AST
inr high
aptt high
thrombin time normal/high
plt count normal/low
bleed time normal/high
ast high
plt defect: INR, APTT, Thrombin time, Plt count, bleed time
all normal apart from high bleed time
vK deficiency: INR, APTT, Thrombin time, Plt count, bleed time
very high inr
high aptt
normal everything else
haemophilia: INR, APTT, Thrombin time, Plt count, bleed time
very high aptt
normal everything else
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