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|Title:||Diagnosis and management of thrombotic thrombocytopenic purpura (TTP) in Australia: findings from the first 5 years of the Australian TTP/thrombotic microangiopathy registry.|
|Epworth Authors:||Cohney, Solomon (Shlomo)|
|Other Authors:||Blombery, Piers|
TTP Registry Steering Committee
|Keywords:||UroRenal, Vascular Clinical Institute, Epworth HealthCare, Victoria, Australia|
Department of Nephrology, Epworth HealthCare, Richmond, Victoria, Australia.
Purpura, Thrombotic Thrombocytopenic
Thrombotic Thrombocytopenic Purpura
ADAMTS13 protein, human
|Citation:||Internal Medicine Journal. 2016 Jan;46(1):71-9.|
|Abstract:||BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy (TMA). In 2009, the Australian TTP/TMA registry was established to collect data on patients presenting with TTP/TMA throughout Australia. AIM: To summarise information on the diagnosis and management of patients with TTP collected in the first 5 years (2009-2014) of the Australian TTP registry. METHODS: Registry data from June 2009 to October 2014 were reviewed. RESULTS: Fifty-seven patients were identified with TTP (defined as ADAMTS13 activity <10%), accounting for 72 clinical episodes. ADAMTS13 inhibitor testing was performed in nine out of 57 patients (16%), reflecting the limited availability of accredited testing facilities. Sixty-seven out of 72 episodes were treated with therapeutic plasma exchange (PEx) using cryodepleted plasma (40% of episodes), fresh frozen plasma (36%) or a mixture (22%). Median exposure to plasma products was 55.9 L. PEx was commenced ≥2 days from stated diagnosis in 15% of episodes. Adverse reactions to PEx were common with documented allergic reactions (including life threatening) in 21% of episodes. Adjunctive immunosuppression was documented in 76% of episodes (corticosteroid 71% and rituximab 39%). Platelet transfusion was administered in 15% of episodes. CONCLUSIONS: Data from the Australian TTP/TMA registry suggest a heterogenous approach to the diagnosis and management of TTP in Australia over the assessed period. These observations highlight areas for improvement and standardisation of practice, including comprehensive diagnostic testing, more immediate access to PEx and a more uniform approach to adjunctive immunosuppression and supportive care.|
|Journal Title:||Internal Medicine Journal|
|Affiliated Organisations:||Department of Haematology, Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia.|
Department of Epidemiology and Preventive Medicine, Monash University, Melbourne, Victoria, Australia.
Fiona Stanley Hospital, Perth, Western Australia, Australia.
Gold Coast University Hospital, Gold Coast, Queensland, Australia.
St Vincent's Hospital, Sydney, New South Wales, Australia.
The Kirby Institute, University of New South Wales, Sydney, New South Wales, Australia.
Monash Medical Centre, Melbourne, Victoria, Australia.
Department of Medicine, University of Melbourne, Parkville, Victoria, Australia.
Departments of Nephrology, Western Hospital, Sunshine/Footscray, Victoria, Australia.
Departments of Nephrology, Royal Melbourne Hospital, Parkville, Victoria, Australia.
|Type of Clinical Study or Trial:||Review|
|Appears in Collections:||UroRenal, Vascular|
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