COAGULATION DEFECTS Flashcards

1
Q

characterized by multiple clotting abnormalities

Vitamin K Deficiency – depresses synthesis of II, VII, IX, X, protein C, protein S

Liver disease/DIC – causes coagulation problems

A

Acquired Coagulation Deficiencies

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2
Q

Caused by problems in the Factor VIII-vWF complex

Deficiency in Factor VIII or Factor VIII procoagulant protein

A

Hereditary Coagulation Deficiencies

Hemophilia A, Factor VIII-vWF complex

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3
Q

required for activation of factor X

A

Factor VIII

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4
Q

multimeric protein that binds to collagen, heparin, platelet membrane glycoproteins Ib-Ix and IIb-IIIa

A

vW Factor

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5
Q

common inherited bleeding disorder (1% of people)

Clinical Signs**
• spontaneous bleeding from mucous membranes
• excessive wound bleeding
• menorrhagia (heavy periods)
• prolonged bleeding time
A

Von Willebrand’s Disease

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6
Q

name this type of Von Willebrand’s Disease –

autosomal dominant - 70% of all cases

o Causes a mild disease
o Reduced penetrance and variable expressivity

A

Type 1 vWD

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7
Q

name this type of Von Willebrand’s Disease –

autosomal dominant, missense mutation

A

Type 2 vWD

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8
Q

name this type of Von Willebrand’s Disease –

– autosomal recessive

o A much more severe disease causing very low levels of vWF
o Testing may resemble hemophilia due to instability of factor VIII

A

Type 3 vWD

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9
Q

caused by low levels of Factor VIII

Cause – X-linked recessive

A

Hemophilia A

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10
Q

factor IX deficiency (“Christmas Disease”)

Cause – X-linked recessive

A

Hemophilia B

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