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

Disclaimer

Overview

Title

Treatment of Thrombotic Thrombocytopenic Purpura

Authoring Organization

Publication Month/Year

July 1, 2020

Last Updated Month/Year

July 10, 2023

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

Supplemental Methodology Resources

Data Supplement, Data Supplement