Conclusions
| Observations on initial presentation |
| Patients with TTP may have no symptoms and a normal physical examination. TTP may first be suspected by unexpected severe thrombocytopenia and anemia on a routine CBC. Patients with TTP may die suddenly, even when they have had only minor symptoms for 1 or several days. Reported ADAMTS13 activity ≥10% (and even normal activity of ≥60%) does not exclude the diagnosis of TTP. Reported ADAMTS13 activity <10% may reveal unsuspected TTP. TTP may initially occur together with an additional disorder. |
| Observations on management during remission |
| ADAMTS13 activity during remission is unpredictable. Patients may have continuously severe ADAMTS13 deficiency during remission for many years without relapse. Patients may have continuously severe ADAMTS13 deficiency during remission for many years and then have normal ADAMTS13 activity for many more years without relapse. Patients may have continuously normal or nearly normal ADAMTS13 activity for many years and then have severe ADAMTS13 deficiency followed by relapse. Rituximab treatment of severe ADAMTS13 deficiency during remission may prevent relapse; however, the appropriate indication for treatment and the appropriate rituximab regimen are uncertain. |
| Observations on initial presentation |
| Patients with TTP may have no symptoms and a normal physical examination. TTP may first be suspected by unexpected severe thrombocytopenia and anemia on a routine CBC. Patients with TTP may die suddenly, even when they have had only minor symptoms for 1 or several days. Reported ADAMTS13 activity ≥10% (and even normal activity of ≥60%) does not exclude the diagnosis of TTP. Reported ADAMTS13 activity <10% may reveal unsuspected TTP. TTP may initially occur together with an additional disorder. |
| Observations on management during remission |
| ADAMTS13 activity during remission is unpredictable. Patients may have continuously severe ADAMTS13 deficiency during remission for many years without relapse. Patients may have continuously severe ADAMTS13 deficiency during remission for many years and then have normal ADAMTS13 activity for many more years without relapse. Patients may have continuously normal or nearly normal ADAMTS13 activity for many years and then have severe ADAMTS13 deficiency followed by relapse. Rituximab treatment of severe ADAMTS13 deficiency during remission may prevent relapse; however, the appropriate indication for treatment and the appropriate rituximab regimen are uncertain. |