Quiz on TTP Pathophysiology • The Blood Project

What protease is deficient in TTP?

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ADAMTS13

What is the normal function of ADAMTS13

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Cleaves ultra-large vWF multimers, which are prothrombotic

What is a vWF string?

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Ultra-large vWF multimers tethered to the endothelial surface

Match the vWF domains with the function

A2

A3

A1

ADAMTS13 binding

Platelet binding

Collagen binding

Correct! Sorry, Incorrect.

Where is vWF primarily synthesized (more than one answer may apply)?

a

Hepatic stellate cells

b

Endothelial cells

c

Rental tubular epithelial cells

d

Megakarytocyes

e

Astrocytes

What is the major cell type that expresses ADAMTS13?

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Hepatic stellate cell

What cellular organelles store vWF?

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Weibel-Palade bodies (endothelial cells), alpha granules (platelets)

True or false: ADAMT13 is released by hepatic stellate cells as an active enzyme.

a

True

b

False

Which vWF domain(s) is/are largely hidden in the circulating globular conformation?

a

A1

b

A2

c

A3

d

None of above

Congenital (hereditary) TTP is:

a

Autosomal dominant

b

Autosomal recessive

c

X linked