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Algorithm for management of hereditary TTP

Algorithm for management of hereditary TTP
This algorithm addresses the use of plasma infusions for individuals with hereditary TTP. It is not appropriate for those with acquired, autoimmune TTP. This approach may be modified once rADAMTS13 becomes available.
TTP: thrombotic thrombocytopenic purpura; rADAMTS13: recombinant ADAMTS13.
* Each person has an individual normal platelet count that may be more than 300,000/microL or <150,000/microL. Thrombocytopenia should be assessed relative to the individual's baseline rather than a laboratory reference range. Every individual with hereditary TTP should have their baseline platelet count determined (when they are asymptomatic). Refer to UpToDate for details.
¶ Refer to inset box for initial plasma dosing. The dose can be rounded to the nearest plasma unit (ie, we do not discard unused plasma). The volume, frequency, and duration may require modification based on the patient's prior experience, clinical status, and other factors. Specialist input (hematology, transfusion medicine) is advised. Pregnant women who are receiving regular (prophylactic) plasma infusions are not expected to have symptoms of hereditary TTP; however, if an exacerbation does occur (eg, initial diagnosis made during the pregnancy) it is treated the same as in a non-pregnant individual.
Δ The severity of persistent symptoms or the frequency of exacerbations that warrant initiation of regular plasma infusions is individualized. Refer to UpToDate for details.
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