8
Treatment
Immune-mediated TTP (iTTP)
➤ For patients with iTTP experiencing a first acute event, the panel
recommends the addition of corticosteroids to therapeutic plasma
exchange (TPE) over TPE alone. (S-VL)
➤ For patients with iTTP experiencing their first acute event, the panel
suggests the addition of rituximab to corticosteroids and TPE over
corticosteroids and TPE alone. (C-VL)
➤ For patients with iTTP experiencing a relapse, the panel recommends
addition of corticosteroids to TPE over TPE alone. (S-VL)
➤ For patients with iTTP experiencing a relapse, the panel suggests the
addition of rituximab to corticosteroids and TPE over corticosteroids
and TPE alone. (C-VL)
➤ For patients with iTTP experiencing an acute event (first event or
relapse) the panel suggests using caplacizumab over not using
caplacizumab. (C-M)
➤ For patients with iTTP who are in remission, but still have low plasma
ADAMTS13 activity with no clinical signs/symptoms, the panel
suggests the use of rituximab over non-use of rituximab for prophylaxis.
(C-VL)
Hereditary or Congenital TTP (cTTP)
➤ For patients with cTTP who are in remission, the panel suggests either
plasma infusion or a watch and wait strategy. (C-VL)
➤ For patients with cTTP who are in remission, the panel suggests against
the use of factor VIII concentrate infusions versus a watch and wait
strategy. (C-VL)
➤ For patients with iTTP who are pregnant and have decreased plasma
ADAMTS13 activity but with no clinical signs/symptoms, the panel
recommends prophylactic treatment over no prophylactic treatment.
(S-VL)
➤ For patients with cTTP who are pregnant, the panel recommends
prophylactic treatment over no prophylactic treatment. (S-VL)
➤ For patients with cTTP who are pregnant, the panel suggests
prophylactic treatment with plasma infusion over FVIII products. (C-VL)
Treatment
