14
Treatment
Statement 13
➤ We did not systematically search and review the evidence on the role
of ADAMTS13 monitoring in TTP patients in remission. Patients
in remission are usually assessed regularly during follow-up
(typically every month for the first 3 months, then every 3 months
for the first year, then every 6 to 12 months if stable). If available,
ADAMTS13 activity is usually measured serially during each follow-
up assessment, and more frequently if levels begin to drop. Durably
stable ADAMTS13 activity close to the lower limit of normal is usually
a reassuring finding. Conversely, patients with persistently low
ADAMTS13 activity (<10 IU/dL or 10% of normal) may be at risk for
relapse, which may be prevented by administration of rituximab.
SECTION III. Other treatment agents for TTP
➤ The following nine statements pertain to the use of TTP treatment
strategies that fall outside of the panel's prespecified PICO
questions.
Statement 14
➤ We did not systematically search and review the evidence on
vincristine for TTP patients. Patients with refractory TTP
unresponsive to standard treatments may be considered for other
immunosuppressive treatments with scant supporting evidence,
including vincristine. In these cases, vincristine 2 mg is usually
administered intravenously, at a slow rate of infusion. Typically, a
single dose is used, as additional doses can cause neurotoxicity and
bone marrow suppression.
Statement 15
➤ We did not systematically search and review the evidence on
cyclosporine A for TTP patients. Patients with refractory TTP
unresponsive to standard treatments may be considered for other
immunosuppressive treatments with scant supporting evidence,
including cyclosporine A. In these cases, cyclosporine A is usually
administered orally or intravenously in varying doses (e.g., 300 mg/
day orally; 2 to 3 mg/kg daily in twice-daily divided doses). The
appropriate duration of therapy is unknown, although administration
for several months followed by tapering has been reported.
