Quiz on TTP - Part 2 • The Blood Project

Management

True or false: TTP is a medical emergency.

a

True

Treatment should begin as soon as possible, preferably within 4-8 hours of initial clinical diagnosis (BSH Grade 1A), regardless of the time of day.

b

False

Management

True or false: One should wait for the results of ADAMTS13 activity levels before instituting therapy.

a

True

b

False

Do not delay treatment for confirmatory ADAMTS13 measurement, especially if the patient has a high clinical suspicion of TTP.

Management

Which of the following should be started as soon as possible in patients with immune (acquired/autoimmune) TTP:

a

Corticosteroids

Administer methylprednisolone (1 g/day IV for 3 days for adults) or high-dose oral prednisolone (for example, 1 mg/kg/day) (BSH Grade 1B; ISTH Strong recommendation, Very low-certainty of evidence) with tapering when sustained elevation in ADAMTS13 activity is achieved.

b

Therapeutic plasma exchange (TPE)

Plasma exchange is the recommended first-line therapy for TTP by professional organizations including British Society for Haematology (BSH), ISTH, and American Society for Apheresis (ASFA).

c

Rituximab

Consider the addition of rituximab to plasma exchange and corticosteroids (ISTH Conditional recommendation, Very low-certainty of evidence), within 3 days of admission (BSH Grade 1B).

d

Cyclosporin

Management

True or false: plasma infusion is an acceptable alternative to TPE in patients with immune (acquired/autoimmune) TTP.

a

True

b

False

TPE is preferred because it not only provides ADAMTS13 to the patient, but it also removes antibodies against ADAMTS13. If plasma exchange is unavailable or will be delayed, consider large plasma infusions (25-30 mL/ kg), if tolerated, in conjunction with pulsed methylprednisolone.

Management

True or false: Therapeutic plasma exchange (TPE) has been shown to improve survival in patients with immune (acquired/autoimmune) TTP.

a

True

Daily plasma exchange has been reported to reduce mortality rates from an estimated 90% to 10%-20%.

b

False

Management

True or false: patients with TTP and a platelet count < 30 x 10⁹/L should receive platelet transfusion.

a

True

b

False

Do not give platelet transfusions (BSH Grade 1B), unless there is life-threatening bleeding.

Management

All clinical practice guidelines (strongly) recommend TPE and corticosteroids as first line therapy for TTP. Which of the following is/are also recommended up front (within days for presentation) for certain patients with TTP?

a

Azathioprine

b

Rituximab

c

Imatinib

d

Vincristine

e

Caplacizumab

Management

What is Caplacizumab?

a

Humanized anti-von Willebrand factor (vWF) single-variable-domain immunoglobulin (nanobody)

Caplacizumab is a humanized anti-von Willebrand factor (vWF) single-variable-domain immunoglobulin (nanobody) that targets the AI domain of vWF, preventing its interaction with the platelet glycoprotein 1b-IX-V

b

Humanized anti-fibrinogen single-variable-domain immunoglobulin (nanobody)

c

BTK inhibitor

d

Recombinant ADAMTS13

Management

Which of the following statements about Caplacizumab is/are true?

a

Does not correct the underlying ADAMTS13 deficiency nor does it eliminate anti-ADAMTS13 autoantibodies.

b

Depletes ADAMTS13 antibodies

c

Should be restricted to patients with refractory or relapsed TTP

d

Negates the need for immunosuppressive treatments such as rituximab and corticosteroids

e

Causes a von Willebrand disease like phenotype

Match the treatment with mechanism of action in TTP

Plasma exchange

Caplacizumab

Corticosteroids

Rituximab

Reduced antibody levels

Inhibition of vWF binding

Management

True or false: Recombinant ADAMTS13 has been studied as a possible therapy in patients with TTP.

a

True

See N Engl J Med 2024 May 2;390(17):1584

b

False

Management

True or false: Hospitalized patients with TTP should receive thromboprophylaxis once platelet count has reached > 50 × 10⁹/L.

a

True

b

False

Quiz on TTP – Part 2