Diagnosis

Treatment

Document Overview

Design and created by Guideline Central in participation with the International Society on Thrombosis and Haemostasis.

Diagnosis of Thrombotic Thrombocytopenic Purpura

International Society on Thrombosis and Haemostasis

Publication Date: Jun 17, 2025

Page Last Updated: May 5, 2026

GRADE Strength of Recommendations and Implications

Grade	Quality of Evidence
High	We are very confident that the true effect lies close to the estimate of the effect.
Moderate	We are moderately confident that the true effect lies close to that of the estimate of the effect. There is a possibility that it is substantially different.
Low	Our confidence that the true effect lies close to that of the estimate of the effect is low. The true effect may be substantially different from the estimate of the effect.
Very low	We have very little confidence in the effect estimate. The true effect is likely to be substantially different from the estimate of effect.
Evidence / Knowledge Gap	Available evidence insufficient to determine true effect.

Grade	Strength of Recommendation
Strong “AGA recommends...”	For the Patient	For the Clinician	For policy makers
Strong “AGA recommends...”	Most individuals in this situation would want the recommended course and only a small proportion would not.	Most individuals should receive the intervention. Formal decision aids are not likely to be needed to help individuals make decisions consistent with their values and preferences.	The recommendation can be adapted as policy or performance measure in most situations.
Conditional (weak) “AGA suggests...”	The majority of individuals in this situation would want the suggested course, but many would not.	Different choices will be appropriate for individual patients consistent with his or her values and preferences. Use shared decision making. Decision aids may be useful in helping patients make decisions consistent with their individual risks, values, and preferences.	Policy making will require substantial debate and involvement of various stakeholders. Performance measures should assess whether decision making is appropriate.
No recommendation “AGA makes no recommendation...”		The confidence in the effect estimate is so low that any effect estimate is speculative at this time.

Document Overview

Document Title: Diagnosis of Thrombotic Thrombocytopenic Purpura

Authoring Society: International Society on Thrombosis and Haemostasis

Document Publication Date: Jun 17, 2025

Page Last Reviewed/Updated: May 5, 2026

Document Type: Guideline

Country of Publication: United States

Full Text Freely Available: Yes

Full Text Guideline: onlinelibrary.wiley.com/doi/10.1111/jth.15006

Source Citation: Zheng XL, Al-Housni Z, Cataland SR, Coppo P, Geldziler B, Germini F, Iorio A, Keepanasseri A, Masias C, Matsumoto M, McCrae KR, McIntyre J, Mustafa RA, Peyvandi F, Russell L, Tarawneh R, Vesely SK; International Society on Thrombosis and Haemostasis. 2025 focused update of the 2020 ISTH guidelines for management of thrombotic thrombocytopenic purpura. J Thromb Haemost. 2025 Jun 17:S1538-7836(25)00360-5. doi: 10.1016/j.jtha.2025.06.002. Epub ahead of print. PMID: 40533296.

Supplemental Implementation Resources

Document Scope, Criteria, and Use Cases

Document Objectives: The evidence-based guidelines of the International Society on Thrombosisand Haemostasis (ISTH) are intended to support patients, clinicians, and other healthcare professionals in their decisions about the initial diagnosis and management ofacute Thrombotic thrombocytopenic purpura (TTP).

Scope: Diagnosis, Management

Diseases/Conditions (MeSH)

D003933 - Diagnosis

D011697 - Purpura, Thrombotic Thrombocytopenic

D003933 - Diagnosis

D011697 - Purpura, Thrombotic Thrombocytopenic

Keywords: ADAMTS13, Guidelines, TTP, diagnosis, thrombosis

Target Patient Population: adult patients with a suspected Thrombotic thrombocytopenic purpura (TTP)

Target Provider Population: Primary care physicians, emergency orcritical care physicians, hematologists, nephrologists, neurologists, pathologists or transfusion medicine specialists, surgeons, obstetricians and gynecologists, as well as hospitalists

Inclusion Criteria: Male, Female, Adult

Health Care Settings: Ambulatory, Hospital, Long Term Care

Intended Users: Nurse, Nurse Practitioner, Physician, Physician Assistant

Recommendation Development Processes & Methodology

Supplemental Methodology Resource: Data Supplement

Number of Source Documents: 133

Includes peer/external review process?: Yes

Includes public comment process?: Yes

Methodologist involvement?: Yes

Patient involvement?: No

Includes multi-disciplinary group?: Yes

Includes systematic review?: Yes

Grades quality of strength of evidence?: Yes

Grades quality of strength of recommendation?: Yes

Discloses funding source?: Yes

Discloses conflicts of interest?: Yes

Includes benefits/harms analysis with recommendations?: No

Diagnosis of Thrombotic Thrombocytopenic Purpura

Diagnosis

Treatment

Immune-mediated TTP (iTTP)

Recommendation 1

Recommendation 2

Recommendation 3

Recommendation 4

Recommendation 5

Recommendation 6

Hereditary or cTTP

Recommendation 7

Recommendation 8

Recommendation 8b

Recommendation 9

Recommendation 10a

Recommendation 10b

COMMENT

SECTION I. Supportive Care

Statement 1

Statement 2

Statement 3

Statement 4

Statement 5

Statement 6

Statement 7

Statement 8

Statement 9

Statement 10

Statement 11

SECTION II. Relapse Prevention

Statement 12

Statement 13

SECTION III. Other treatment agents for TTP

Statement 14

Statement 15

Statement 16

Statement 17

Statement 18

Statement 19

Statement 20

Statement 21

Statement 22

SECTION IV. TTP and Women’s Health

Statement 23

Statement 24

Statement 25

Statement 26

Statement 27

Statement 28

Statement 29

Statement 30

Statement 31

Statement 32

SECTION V. Refusal of Blood Products

Statement 33

GRADE Strength of Recommendations and Implications

Document Overview

Supplemental Implementation Resources

Document Scope, Criteria, and Use Cases

Recommendation Development Processes & Methodology