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

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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)

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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