Thrombotic Microangiopathy |(TMA) | Microthrombotic Disease

Flowcharts For The Diagnosis, Treatment, And Outpatient Follow Up Of Patient With iTTP

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)

START PLASMA EXCHANGE (PEX)

1.0 – 1.5 plasma volumes daily

CORTICOSTEROIDS

PREDNISONE

1 mg/Kg/day (max dose 100 mg)

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

Weekly for 1 month,

Monthly x 3 months and then, every 3 months

ADAMTS-13

< 10% after 28

days of therapy

ADAMTS-13

> 10%

2 consecutive weeks

Discontinue

Caplacizumab