Home
FAST4TMA
About Us
Physician Resources
Patient Resources
Shop
Contact Us
More
Testing
TMA: Coombs negative hemolytic anemia + Thrombocytopenia
Send citrate tube and ADAMTS-13 activity, antibody or inhibitor titer
Additional testing: DIC panel, Troponins, Hepatitis B panel and pregnancy test
Pediatric population see text
If hemodynamically stable
ICU admission is NOT required
(follow institutional guidelines)
Initiate Treatment Immediately
Initiate therapy, DO NOT wait for ADAMTS 13 activity results
START PLASMA EXCHANGE (PEX)
1.0 – 1.5 plasma volumes daily
CORTICOSTEROIDS
PREDNISONE
1 mg/Kg/day (max dose 100 mg)
Consider the use of CAPLACIZUMAB if HIGH suspicion or confirmed iTTP WITHOUT bleeding
Consider upfront RITUXIMAB 375 mg/m2 weekly X 4 weeks for new onset or
In the relapsed setting without previous Rituximab administration
Platelet count > 150, 000 x 2 consecutive days, THEN
Stop PEX and taper steroids within 4 weeks
VISITS AND STANDARD LABS
ADAMTS-13 activity
On Caplacizumab
Not on Caplacizumab
Weekly while on caplacizumab
Weekly for 1 month,
Monthly x 3 months and then, every 3 months
ADAMTS-13
< 10% after 28
days of therapy
> 10%
2 consecutive weeks
Consider extending Caplacizumab and intensify immunosuppression
Discontinue
Caplacizumab