Algorithm for management of hereditary TTP

This algorithm addresses the use of ADAMTS13 replacement with plasma or recombinant ADAMTS13 for individuals with hereditary TTP. It is not appropriate for those with immune TTP. If both forms of replacement are available, we suggest recombinant ADAMTS13 over plasma. However, plasma may be an appropriate choice for some patients depending on availability, patient preference, and cost. Shared decision-making is encouraged.

TTP: thrombotic thrombocytopenic purpura.

* Each person has an individual normal platelet count that may be >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 for dosing. 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 individuals who are receiving prophylaxis are not expected to have symptoms of hereditary TTP; however, if an exacerbation does occur during pregnancy, it is treated the same as in non-pregnant individuals.

Δ The severity of persistent symptoms or the frequency of exacerbations that warrant initiation of prophylaxis is individualized. Refer to UpToDate for details.

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Algorithm for management of hereditary TTP