HUS/TTP

Question 1

If you are commencing a patient on eculizumab in the acute setting (i.e. aHUS), what is recommended to protect against N meningitidis infection?

Answer 1

Vaccination and 2 weeks of antibiotic prophylaxis (with ciprofloxacin).

Question 2

What ADAMTS13 activity is suggestive of TTP?

Answer 2

<10%

Question 3

What if the ADAMTS13 level is greater than 10% and the patient is STEC negative?

Answer 3

Consider aHUS or Secondary TMA

Question 4

What features (on labs) go against a diagnosis of TTP?

Answer 4

Plts >30,000 and Creat > 2.25mg/dl

Question 5

What percentage of patients with STEC-HUS develop renal failure requiring dialysis?

Answer 5

60%

Question 6

What is the most severe genetic mutation resulting in aHUS?

Answer 6

Mutations involving Factor H

Question 7

What genetic mutation portends the best prognosis in transplanted individuals with aHUS?

Answer 7

Mutations in MCP (this is an endothelial protein)

Question 8

Apart from eculizumab and kidney transplant, is there any other measure that might reduce risk of recurrent kidney disease in aHUS?

Answer 8

Combined liver and kidney transplant

Question 9

aHUS secondary to DGKE mutations (in children < 1yr) tends to recur after kidney transplant. True/False

Answer 9

False

Question 10

Upshaw-Shulman Syndrome is a cause for TTP. True/False

Answer 10

True… these flashcards are about HUS/TTP… this is caused by a genetic mutation and functional deficiency of ADAMTS13.

Question 11

What is the mortality rate for TTP without plasma exchange?

Answer 11

90%

Question 12

What is the best treatment for relapsing or resistant cases of TTP? Assuming patient is already on eculizumab

Answer 12

Add rituximab to the regimen.