Algorithm for tapering therapy after an initial response in a patient with immune thrombotic thrombocytopenic purpura (TTP)

This algorithm outlines our approach to tapering therapy in an individual with immune TTP who is receiving therapy and has a documented platelet count response, defined as a platelet count ≥150,000/microL for 2 days or a platelet count plateau (normal or supranormal) for 3 days. Refer to UpToDate topics on treatment of acquired autoimmune TTP and management following recovery from an acute episode of TTP for additional details about monitoring and interventions.

TTP: thrombotic thrombocytopenic purpura; TPE: therapeutic plasma exchange; CBC: complete blood count; LDH: lactate dehydrogenase.

* Defined as a platelet count ≥150,000/microL for at least 2 days or a stable platelet count plateau in the normal or supranormal range for 3 days. Refer to UpToDate for details.

¶ Daily assessment of clinical status, CBC, and LDH is done for approximately 2 to 3 days after stopping TPE; monitoring is then extended slowly, with the exact timing dependent on clinical status.

Δ Individuals who have a recurrence within a month of the acute episode are considered to have refractory disease and are treated more aggressively. Those who have a recurrence >1 month after the acute episode are considered to have a relapse and are treated for a new episode, similarly to the previous episode. Refer to the UpToDate algorithm on initial treatment of TTP and the UpToDate topic on treatment of refractory or relapsed TTP for details of management.

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Algorithm for tapering therapy after an initial response in a patient with immune thrombotic thrombocytopenic purpura (TTP)