Treatment of Thrombotic Thrombocytopenic Purpura
Publication Date: July 1, 2020
Last Updated: March 14, 2022
Recommendations
iTTP first event
For patients with iTTP experiencing a first acute event, the panel recommends the addition of corticosteroids to therapeutic plasma exchange (TPE) over TPE alone. (Very Low, Strong)
612
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. (Very Low, Conditional (weak))
612
iTTP relapsing episode
For patients with iTTP experiencing a relapse, the panel recommends addition of corticosteroids to TPE over TPE alone. (Very Low, Strong)
612
For patients with iTTP experiencing a relapse, the panel suggests the addition of rituximab to corticosteroids and TPE over corticosteroids and TPE alone. (Very Low, Conditional (weak))
612
iTTP first or relapsing episode
For patients with iTTP experiencing an acute event (first event or relapse), the panel suggests using caplacizumab over not using caplacizumab. (Moderate, Conditional (weak))
612
iTTP in remission
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 nonuse of rituximab for prophylaxis. (Very Low, Conditional (weak))
612
cTTP in remission
For patients with cTTP who are in remission, the panel suggests either plasma infusion or a watch and wait strategy. (Very Low, Conditional (weak))
612
For patients with cTTP who are in remission, the panel suggests against the use of factor VIII (FVIII) concentrate vs. a watch and wait strategy. (Very Low, Conditional (weak))
612
TTP during pregnancy
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. (Very Low, Strong)
612
For patients with cTTP who are pregnant, the panel recommends prophylactic treatment over no prophylactic treatment. (Very Low, Strong)
612
For patients with cTTP who are pregnant, the panel suggests prophylactic treatment with plasma infusion over FVIII products. (Very Low, Conditional (weak))
612
Recommendation Grading
Overview
Title
Treatment of Thrombotic Thrombocytopenic Purpura
Authoring Organization
International Society on Thrombosis and Haemostasis
Publication Month/Year
July 1, 2020
Last Updated Month/Year
February 6, 2024
Supplemental Implementation Tools
Document Type
Guideline
External Publication Status
Published
Country of Publication
US
Inclusion Criteria
Female, Adult
Health Care Settings
Ambulatory
Intended Users
Physician, nurse, nurse practitioner, physician assistant
Scope
Management, Treatment
Diseases/Conditions (MeSH)
D011697 - Purpura, Thrombotic Thrombocytopenic
Keywords
rituximab, treatment guidelines, TTP, caplacizumab, Thrombotic, Thrombocytopenic Purpura