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Thrombotic Thrombocytopenic Purpura: 10 Year Analysis of the United Kingdom TTP Registry
ISTH Academy. Shin J. 07/08/19; 273939; OC 46.3 Topic: ADAMTS13 & TTP
Jin-Sup Shin
Jin-Sup Shin
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OC 46.3

Thrombotic Thrombocytopenic Purpura: 10 Year Analysis of the United Kingdom TTP Registry

J.-S. Shin1,2, F. Alwan1,2, M. Scully1,2, UK TTP Group
1University College London Hospitals NHS Foundation Trust, Department of Haematology, London, United Kingdom, 2University College London, Haemostasis Research Unit, London, United Kingdom

Main Topic: Prothrombotic States
Category: ADAMTS13 & TTP

Background: Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy, confirmed by ADAMTS13 activity < 10%).
Aims: Epidemiological analysis of the UK TTP registry.
Methods: UK study of patients presenting with acute TTP between 2009 and 2018.
Results: Out of 1087 acute TTP episodes affecting 663 patients, 475 (73% female, 27% male) were consented to the registry. Median age at presentation was 43 years (range 9-93). 60% were Caucasian, 22% Afro-Caribbean. 84% had immune-mediated TTP (idiopathic 76%; secondary 24%) and 16% had congenital TTP. From 564 episodes, relapse rate was 19%.
Commonest presenting findings were neurological (71%), raised troponin (55%) and bleeding/petechiae (48%). Of the 45% admitted to the intensive care unit, 10% required intubation. There was no difference in presenting features between idiopathic and secondary TTP.
At presentation, 92% had ADAMTS13 activity < 10% and 25% had platelet counts < 10 x109/l (Table 1). 81% received steroids and a median of 11 (range 1-75) plasma exchanges (PEXs) (Table 2). 74% received rituximab, at a median of 3 days after admission and median of 4 doses (range 1-10). 44% of patients had platelets < 150 x109/l at 7 days, compared to 4% at 1 month. Normalisation of ADAMTS13 occurred in 61% of patients by 1 month, with 79% achieving at least a partial response (ADAMTS13 ≥30%). 13% of cases attained haematological remission but had ADAMTS13 persistently < 10%. Median time to relapse was 26 months (range 1-114) and 61% were successfully re-treated with Rituximab.
89 patients received Rituximab prophylaxis to prevent acute relapse. The true number is likely to be higher, as this data was not initially collected in the registry.
Conclusions: TTP is a heterogeneous condition. Number of PEXs to remission appears to have decreased over the years (Table 2). Although Rituximab usage in the acute setting remains consistent, its use prophylactically has increased.


  Percentage of patients
  At diagnosis Day 7 1 month 3 months
plt <10 x10 9/L 27% 2% 0% 0%
plt <50 x10 9/L 86% 15% 0% 1%
plt <150 x10 9/L 98% 44% 4% 5%
plt >150 x10 9/L 2% 56% 96% 95%
ADAMTS13: 0-10 % 92% 58% 13% 14%
ADAMTS13: 0-30 % 97% 67% 20% 21%
ADAMTS13: 30-60 % (PR) 2% 14% 18% 12%
ADAMTS13: ≥60 % (CR) 0% 16% 61% 67%
[Change in platelet count and ADAMTS13 activity after treatment]




  PEX usage: Median (range) % of acute cases where Rituximab was used
2009-2010 14 (2-75) 72%
2011-2012 12.5 (1-74) 69%
2013-2014 11 (4-51) 76%
2015-2016 10 (3-45) 75%
2017-2018 8 (3-65) 78%
Overall 11 (1-75)  
[Comparison of acute TTP treatment by year-group]

OC 46.3

Thrombotic Thrombocytopenic Purpura: 10 Year Analysis of the United Kingdom TTP Registry

J.-S. Shin1,2, F. Alwan1,2, M. Scully1,2, UK TTP Group
1University College London Hospitals NHS Foundation Trust, Department of Haematology, London, United Kingdom, 2University College London, Haemostasis Research Unit, London, United Kingdom

Main Topic: Prothrombotic States
Category: ADAMTS13 & TTP

Background: Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy, confirmed by ADAMTS13 activity < 10%).
Aims: Epidemiological analysis of the UK TTP registry.
Methods: UK study of patients presenting with acute TTP between 2009 and 2018.
Results: Out of 1087 acute TTP episodes affecting 663 patients, 475 (73% female, 27% male) were consented to the registry. Median age at presentation was 43 years (range 9-93). 60% were Caucasian, 22% Afro-Caribbean. 84% had immune-mediated TTP (idiopathic 76%; secondary 24%) and 16% had congenital TTP. From 564 episodes, relapse rate was 19%.
Commonest presenting findings were neurological (71%), raised troponin (55%) and bleeding/petechiae (48%). Of the 45% admitted to the intensive care unit, 10% required intubation. There was no difference in presenting features between idiopathic and secondary TTP.
At presentation, 92% had ADAMTS13 activity < 10% and 25% had platelet counts < 10 x109/l (Table 1). 81% received steroids and a median of 11 (range 1-75) plasma exchanges (PEXs) (Table 2). 74% received rituximab, at a median of 3 days after admission and median of 4 doses (range 1-10). 44% of patients had platelets < 150 x109/l at 7 days, compared to 4% at 1 month. Normalisation of ADAMTS13 occurred in 61% of patients by 1 month, with 79% achieving at least a partial response (ADAMTS13 ≥30%). 13% of cases attained haematological remission but had ADAMTS13 persistently < 10%. Median time to relapse was 26 months (range 1-114) and 61% were successfully re-treated with Rituximab.
89 patients received Rituximab prophylaxis to prevent acute relapse. The true number is likely to be higher, as this data was not initially collected in the registry.
Conclusions: TTP is a heterogeneous condition. Number of PEXs to remission appears to have decreased over the years (Table 2). Although Rituximab usage in the acute setting remains consistent, its use prophylactically has increased.


  Percentage of patients
  At diagnosis Day 7 1 month 3 months
plt <10 x10 9/L 27% 2% 0% 0%
plt <50 x10 9/L 86% 15% 0% 1%
plt <150 x10 9/L 98% 44% 4% 5%
plt >150 x10 9/L 2% 56% 96% 95%
ADAMTS13: 0-10 % 92% 58% 13% 14%
ADAMTS13: 0-30 % 97% 67% 20% 21%
ADAMTS13: 30-60 % (PR) 2% 14% 18% 12%
ADAMTS13: ≥60 % (CR) 0% 16% 61% 67%
[Change in platelet count and ADAMTS13 activity after treatment]




  PEX usage: Median (range) % of acute cases where Rituximab was used
2009-2010 14 (2-75) 72%
2011-2012 12.5 (1-74) 69%
2013-2014 11 (4-51) 76%
2015-2016 10 (3-45) 75%
2017-2018 8 (3-65) 78%
Overall 11 (1-75)  
[Comparison of acute TTP treatment by year-group]

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