TTP Quiz – Part 4

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TTP is a thrombotic microangiopathy caused by a deficiency of ___ which results in thrombocytopenia, microangiopathic hemolytic anemia, and widespread multiorgan thrombosis and injury.

TTP is also known by eponym.

Congenital TTP is also known by eponym.

True or false: all types of thrombotic microangiopathy are characterized by ADAMTS13 activity level < 10%.

a
True
b
False
c
Patients with CKD
d
Black persons
e
Adults aged 18-50 years

Congenital TTP is reported to account for what % of all TTP cases:

a
1%
b
5%
c
10%
d
25%
e
50%

TTP is more common is which populations/groups of patients (more than one answer may apply):

a
Female adults
b
Infants
c
10%
d
25%
e
50%

What is the annual incidence of TTP in the United States?

a
3 per 1,000
b
3 per 10,000
c
3 per 100,000
d
3 per million
e
3 per 10 million

True or false: the clinical expression of ADAMTS13 deficiency may require a triggering event.

a
True
e.g., connective tissue diseases, pregnancy, HIV infection, drugs
b
False

Which statement is/are true:

a
vWF contains multiple ADAMTS13-specific cleavage sites
b
ADAMTS13 contains multiple vWF-specific cleavage sites
c
Both A and B

The historically characterized “clinical pentad” of immune TTP is present in what % of patients:

a
1%
b
5-10%
c
25%
d
50%
e
75%

Schistocytes
Valve hemolysis
MAHA
Organ dysfunction
Hemolytic anemia
Thrombocytopenia
March hemoglobinuria
MAHA
TMA
MAHA alone & TMA

Neurologic symptoms are reported in about what % of patients with TTP:

a
1%
b
5-10%
c
25%
d
50%
e
75%

Suspect diagnosis of TTP in patients with (more than one answer may apply):

a
Acute renal failure
b
Isolated MAHA and thrombocytopenia
c
New focal neurological symptoms + MAHA
d
Prior history of TTP

True or false: blood sample for ADAMTS13 activity levels should be obtained prior to therapeutic plasma exchange.

a
True
b
False

True or false: therapy for TTP should wait until ADAMTS13 activity levels are known to be low.

a
True
b
False

Assuming that we should start therapy preemptively in those at high suspicion for having TTP (before the ADAMTS13 activity results are known), how do we decide who should be treated?

Which of the following tests should be carried out in a patient with suspected TTP:

a
Creatinine
b
Troponin
c
Haptoglobin
d
LDH
e
Peripheral smear

Creatinine
ALT
Haptoglobin
Indirect bilirubin
Direct bilirubin
Plasma hemoglobin
AST
Troponin
CK
LDH
Hemolytic marker
Not a hemolytic marker

True or false: TTP should be treated as a medical emergency.

a
True
b
False

According to clinical practice guidelines, treatment should begin as soon as possible, preferably within ___ hours of initial clinical diagnosis.

What are the two treatments that should be started as soon as possible, based on strong recommendations from clinical practice guidelines?

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