Firm Conference 2/21/25

Prev
 1 / 12 
Next
Prev
 1 / 12 
Next

What do mean by the term thrombotic microangiopathy (TMA) (choose the be best answer)?

a
Microangiopathic hemolytic anemia (MAHA)
This may occur with conditions other than TMA (think valve hemolysis)
b
MAHA + thrombocytopenia
Practically speaking, patients are often diagnosed based on these 2 criteria because there is no overt evidence of organ dysfunction
c
MAHA + thrombocytopenia + organ dysfunction
d
Hemolytic anemia
MAHA is one of many causes of hemolytic anemia

Which statement is true:

a
TMA is a type of TTP
b
TTP is a type of TMA

Regarding TTP, how common is the classic pentad of fever, thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, kidney impairment and fever?

a
1%
b
5-10%
Often the diagnosis is based on MAHA and thrombocytopenia +/- neurological symptoms
c
25%
d
50%
e
75%

What is the normal function of ADAMTS13?

a
Cleaves fibrinogen
b
Kills platelets
c
Cleaves large von Willebrand factor multimers
Yes! Cleavage results in smaller, less procoagulant multimers, which protects against excessive platelet binding and platelet-mediated occlusion of blood vessels
d
Inhibits platelet-platelet aggregation

ADAMTS13 activity level is a send out test. It takes several days to come back. How do we determine which patients to treat empirically for TTP while waiting for the result?

What is more common:

a
Hereditary TTP
b
Immune TTP
95% of cases

What is the treatment of choice for first episode of immune TTP (more than one answer may apply).

a
Therapeutic plasma exchange (plasmapheresis)
b
Cyclosporine
c
Corticosteroids
d
Caplacizumab
Clinical practice guidelines are beginning to suggest Caplacizumab up front, but these are conditional recommendations vs. STRONG recommendations for TPE and steroids
e
Splenectomy

True of false: therapeutic plasma exchange is indicated for TTP, but not for other types of TMA.

a
True
If ADAMTS13 levels return > 20% in a patient with TMA, TPE is generally discontinued
b
False

At the BIDMC, what is true regarding care of patient with suspected TTP?

a
Hematology consult service involved, which oversees TPE
b
Pheresis service involved, which covers all aspects of heme care
c
Both hematology and pheresis services involved
Prev
 1 / 12 
Next