HUS and TTP

Question 1

symptoms of HUS

Answer 1

prodromal illness of diarrhea, abdominal pain, vomiting

Question 2

laboratory findings of HUS (classic triad)

Answer 2

MAHA (Hgb<8) negative Coomb’s test, schistocytes thrombocytopenia <140K usually 40K AKI also see high LDH, high bilirubin, and low haptoglobin

Question 3

when do we see HUS?

Answer 3

usually after diarrhea illness from shiga toxin producing enterohemorrhagic E coli.

Question 4

who gets HUS?

Answer 4

seen more in kids but can be seen in adults and similar presentation to TTP but has more renal and less neurological symptoms as TTP

Question 5

do we give pts with HUS antibiotics even if they have fever?

Answer 5

no. it can worsen their disease as the E COLI die they release more shiga toxin also can put pt in renal failure.

Question 6

which is the best way to diagnose HUS

Answer 6

clinically made with MAHA, thrombocytopenia and AKI serological testing for E coli O157 H7 which is more sensitive. Stool cultures not helpful because the bacteria may be present in stool only for a few days after the onset of symptoms.

Question 7

what causes TTP?

Answer 7

this is from the formation of small vessel, platelet rich, thrombi due to severe deficiency ADAMTS13, which helps in cleave vWF protein. This then can stop the microthrombi from forming. associated with pregnancy due to formation of autoantibodies (acquired) or the emergence of a previously unknown hereditary case.

Question 8

TTP classically has:

Answer 8

severe thrombocytopenia <10K MAHA - schistocytes on blood smear organ dysfunction: thrombi induced ischemia which can lead to neurological manifestations (confusion and headache and mild renal insufficiency) Cr<2 usually

Question 9

what do we do to diagnose TTP?

Answer 9

normal PT/INR (rules out DIC) negative Coomb’s testing ADAMTS13 levels <10% —testing takes a whilet to come back so we treat empirically based upon level of suspicion with PLASMIC score

Question 10

what is the PLASMIC score

Answer 10

predictive score for probability for TTP based on platelet count, hemolysis, active cancer, age, history of stem cell transplant, MCV, INR and Cr. if low risk score<4 - think other causes if intermediate risk 5- send ADAMST13 testing and keep close observation and consider plasma exchange if no other causes If high risk >6- send for ADAMST13 and get expert opinion and immediate plasma exchange.

Question 11

Treatment of TTP to prevent death

Answer 11

plasma exchange, steroids, and rituximab

Question 12

Comparison of TTP, HUS, and ITP

Answer 12