Predicting bleeding risk in people with congenital bleeding disorders using novel tests that measure both clot formation and clot breakdown.

Predicting bleeding in patients with either hemophilia, fibrinolytic disorders or BDUC using an integrated thrombin and plasmin generation approach. - Thrombin and Plasmin generation profiling in congenital bleeding disorders

Status

Active, not recruiting

Phases

Unknown

Study type

Observational

Source

NL-OMON

Registry ID

NL-OMON58478

Enrollment

250

Registered

2025-11-03

Start date

2026-01-01

Completion date

Unknown

Last updated

2026-07-13

For informational purposes only — not medical advice. Sourced from public registries and may not reflect the latest updates. Terms

Conditions

Hemophilia, Fibrinolytic disorders, Bleeding Disorder of unknown cause (BDUC) Hemophilia, Fibrinolytic disorders, Bleeding Disorder of unknown cause (BDUC)

Interventions

None listed

Eligibility

Age

18 Years to 99 Years

Inclusion criteria

Inclusion criteria: Patients aged 18 years and older with hemophilia A or B, currently receiving treatment at the Hemophilia Treatment Center Nijmegen-Eindhoven-Maastricht, Nijmegen location. Patients with severe (<1 IU/dL FVIII or FIX), moderate (1-5 IU/ dL FVIII or FIX), or mild (>5-40 IU/dL FVIII or FIX) hemophilia are eligible for inclusion.  Hemophilia carriers aged 18 years and older, currently receiving treatment at the Hemophilia Treatment Center Nijmegen-Eindhoven-Maastricht, Nijmegen location. Carriers with a confirmed hemophilic mutation are eligible for inclusion. Patients aged 18 years and older with a fibrinolytic disorder, currently receiving treatment at the Hemophilia Treatment Center of Nijmegen-Eindhoven Maastricht, Nijmegen location. A fibrinolytic disorder is defined as: PAI-1 deficiency: PAI-1 antigen level below the lower limit of normal (< 3.4 ng/ml), or; Hyperfibrinolysis: either an elevated ECLT ratio (before and after venous compression) > 5.6 or a ECLT before venous compression of < 116 min.   Patients aged 18 years and older diagnosed with a BDUC, currently receiving treatment at the Hemophilia Treatment Center of Nijmegen-Eindhoven-Maastricht, Nijmegen location. A bleeding disorder of unknown cause is defined by two main criteria: an increased bleeding tendency, either based on the physician’s clinical assessment and/ or an elevated clinical bleeding score (ISTH BAT), and the absence of abnormalities in available laboratory tests. 

Exclusion criteria

Exclusion criteria: No informed consent provided Age < 18 years old Presence of a concomitant bleeding disorder

Design outcomes

Primary

Measure	Time frame
The primary endpoint is the association between: Thrombin and plasmin generation profiles,  parameters reflecting the severity of the bleeding disorder, such as factor (F)VIII or FIX activity levels, PAI-1 antigen, a2AP activity levels, tPA levels, and euglobulin clot lysis time, and the clinical bleeding phenotype (ISTH BAT score, bleeding severity, annual bleeding rate, additional factor concentrate, joint status, age and reason for diagnosis).	—

Measure

Time frame

The primary endpoint is the association between: Thrombin and plasmin generation profiles,  parameters reflecting the severity of the bleeding disorder, such as factor (F)VIII or FIX activity levels, PAI-1 antigen, a2AP activity levels, tPA levels, and euglobulin clot lysis time, and the clinical bleeding phenotype (ISTH BAT score, bleeding severity, annual bleeding rate, additional factor concentrate, joint status, age and reason for diagnosis).

—

Secondary

Measure	Time frame
Additionally, the study will examine the relationship between thrombin and plasmin generation and the quality of life.	—

Countries

Netherlands

Contacts

Public ContactSEM Schols

Radboud Universitair Medisch Centrum

saskia.schols@radboudumc.nl0243618823

Outcome results