Quiz on TTP – Part 3

Prev
 1 / 46 
Next
Prev
 1 / 46 
Next

Congenital TTP is reported to account for about what percentage of all TTP cases?

a
1%
b
5%
c
10%
d
50%
e
75%

Congenital TTP is reported to account for a higher percentage of TTP cases in which populations (vs. general population)?

a
Black persons
b
Children
c
Patients with CKD
d
Pregnant women

What is the annual incidence of TTP in the US?

a
3.1 per hundred persons
b
3.1 per thousand persons
c
3.1 per 100,000 persons
d
3.1 per million persons

Acquired TTP is caused by:

a
Alloantibodies against ADAMTS13
b
Autoantibodies against vWF
c
Mutations in ADAMTS13 gene
d
Autoantibodies against ADAMTS13

ADAMTS13-mediated proteolysis of vWF (more than one answer may apply):

a
Reduces the multimer size of vWF
b
Increases the multimer size of vWF
c
Limits platelet adhesion and aggregation
d
Causes destruction of platelets

True or false: acute kidney failure is never seen in TTP

a
True
b
False

True or false: neurological findings are common in patients with TTP

a
True
b
False

Hereditary TTP is diagnosed based on which of the following (more than one answer may apply):

a
Reduced plasma ADAMTS13 activity (< 10%)
b
Presence of anti-ADAMTS13 antibodies
c
Absence of anti-ADAMTS13 antibodies
d
Presence of a homozygous or compound heterozygous ADAMTS13 variant in genetic screening

Testing in patients suspected of having immune TTP includes:

a
CBC
b
Creatinine
c
Peripheral smear
d
Cardiac troponin
e
Haptoglobin

What lab tests are typically abnormal in a patient with TTP:

a
Hemoglobin
b
Platelet count
c
LDH
d
Creatinine
e
Haptoglobin

True or false: ADAMTS13 activity levels should be determined using pretreatment blood samples.

a
True
b
False

True or false: TTP should be considered a medical emergency.

a
True
b
False

True or false: Treatment should be withheld until the results of ADAMTS13 activity come back.

a
True
b
False

True or false: TTP is an absolute contraindication for platelet transfusions.

a
True
b
False
May transfuse for life-threatening bleeding.

True or false: TPE is not used in patients with secondary TTP.

a
True
b
False

True or false: plasma infusion may be used instead of TPE in patients with hereditary TTP.

a
True
Though sometimes TPE is used to control plasma volume and to remove UL-vWF
b
False

True or false: thromboprophylaxis should not be given to patients with TTP.

a
True
b
False
Give thromboprophylaxis once platelet counts are > 50 × 109/L in hospitalized/immobilized patients,

What is the effect of therapeutic plasma exchange on TTP mortality?

a
Reduction from 90% to 50%
b
Reduction from 90% to 10%
c
Reduction from 90% to 30%
d
Never studied in a clinical trial

According to British Society for Standards in Haematology (BSH) recommendations, after initial clinical diagnosis of TTP, therapeutic plasma exchange should be started within:

a
30 minutes
b
4-8 hours
c
24 hours
d
2 days

A patient is started on therapeutic plasma exchange because of a high suspicion of TTP, pending ADATMS13 activity (send out lab). The result comes back 3 days later. TPE can be safely stopped if ADAMTS13 activity is (more than one answer may apply):

a
5%
b
10%
c
20%
d
30%

True or false: If plasma exchange is unavailable or will be delayed, large plasma infusions (25-30 mL/ kg) can be administered if tolerated.

a
True
b
False

Corticosteroids are usually given:

a
Prior to TPE
b
During TPE
c
Following TPE

The medium time to response to rituximab is:

a
1 day
b
10 days
c
20 days

What statement(s) about caplacizumab is/are correct:

a
Caplacizumab does not correct the underlying ADAMTS13 deficiency
b
Caplacizumab does not liminate anti-ADAMTS13 autoantibodies
c
Caplacizumab may cause a von Willebrand disease phenotype
d
Immunosuppressive treatments such as rituximab and corticosteroids are still needed to manage the underlying disease process

What statement(s) about caplacizumab is/are correct:

a
Given once daily, 11 mg subcutaneous injection
b
After the plasma exchange treatment period, administered 11 mg of caplacizumab by subcutaneous injection once daily for 30 days
c
In patients weighing < 40 kg, consider a 5 mg/day dosing.

Prev
 1 / 46 
Next