PBI's founder Professor Ross Baker, recently attended the 30th ISTH congress in London, leading several key presentations on bleeding disorders.  As a follow-up to the event, we're excited to share with you a quick summary of his presentation on standardising testing for Thrombotic Thrombocytopenic Purpura (TPP).

TTP is a rare and life-threatening disorder if not diagnosed quickly. One of the key laboratory tests used in diagnosis is measuring the ADAMTS13 protein activity. ADAMTS13 is a vital anticoagulant protein and when deficiencies occur microthrombi form and results in TTP.

Currently there are other ADAMTS13 tests available, however the aim of the IMATAS study is to address and overcome the following issues which are central to diagnosing TTP:

• Lack of international gold standard for selected investigative procedures (assays)
• Accurate measurement of antigen and antibody activity
• Consensus on laboratory methods 
• Result interpretation  

Phase 1 of the IMATAS collaborative study enlisted 16 leading global laboratories and experts in TTP to measure real world samples prepared under controlled conditions. Although excellent in measuring ADAMTS13 activity, phase 1 of the study found that variations exist in reporting units, and also in the measurements of antibodies - also important for TTP diagnosis.

IMATAS highlights the need for additional WHO standards in addition to antibody and antigen activity to bridge the knowledge gap in TTP diagnosis. Early detection and accurate classification of people with TTP is crucial as time is everything in regard to the formation of microthrombi; which can lead to organ damage and death. This study demonstrated the need to improve diagnosis of this disease and that ADAMTS13 activity and antibody testing needs to be faster and more accurate.

The Perth Blood Institute is taking a lead role in this space with the IMATAS collaborative study; as a central site for study co-ordination and management through Prof. Baker and his research team.