QUANTIFICATION OF IGG ANTI-ADAMTS13 ANTIBODY LEVEL CAN PREDICT THE LIKELIHOOD OF MORTALITY IN ACUTE THROMBOTIC THROMBOCYTOPENIC PURPURA
(Abstract release date: 05/19/16)
EHA Library. Alwan F. 06/12/16; 135321; S827
Dr. Ferras Alwan
Contributions
Contributions
Abstract
Abstract: S827
Type: Oral Presentation
Presentation during EHA21: On Sunday, June 12, 2016 from 09:00 - 09:15
Location: Room H4
Background
Acute Idiopathic Thrombotic Thrombocytopenic Purpura (TTP) is a life threatening disorder caused by inhibition of the Von Willebrand factor cleaving protein ADAMTS13 by acquired antibodies, usually of the IgG subclass. Its prevalence has been estimated at four to six cases per million per year and is characterized by hemolytic anemia, thrombocytopenia, fever, neurological symptoms and renal dysfunction. Untreated, mortality has been documented at 90% but even with treatment, it remains around 15-20%. Plasma Exchange (PEX) is the mainstay of treatment but immunosuppressive/immunomodulatory therapy is often also required. .Since January 2009, the United Kingdom TTP registry has been collecting information on all acute presentations of TTP across the country. In addition to testing for ADAMTS13 activity, confirmation of the presence of Anti-ADAMTS 13 IgG antibodies forms part of the initial work up.
Aims
A retrospective review to evaluate whether the presenting IgG ADAMTS13 antibody level can predict the likelihood of mortality in acute presentations of TTP.
Methods
360 acute events were identified from the registry involving 314 patients treated in 63 hospitals. Only acquired TTP was considered with all cases of congenital TTP excluded. Acute TTP was defined as ADAMTS13 protease activity below 10% (FRETS vWF-73 assay, NR: 60-120%) or between 10-20% with a detectable IgG ADAMTS13 antibody present. Acute events were then stratified into groups depending on the presenting IgG ADAMTS13 antibody level (0-29%, 30-59%, 60-89%, >90%).
Results
Of the events identified, 68% of those involved were female with a median age of 46 (range 5-90 years) and a median ADAMTS13 at diagnosis of <5% (range <5% – 57%). There was no statistically significant difference when considering these factors between the subgroups. 40 deaths were noted in total, accounting for 11.1% of all events. There was a statistically significant increase in mortality in patients with a presenting IgG ADAMTS13 antibody level above 30% compared to those with a level below 30% (p=0.0023). Subgroup analysis of those presenting with an IgG ADAMTS13 antibody level above 30% found no significant difference in mortality if the level was 30-59%, 60-89% or above 90% (p=0.83).
Conclusion
There is an increased risk of mortality seen in patients with acute TTP and a presenting IgG ADAMTS13 antibody level above 30%. Such patients should be considered for more aggressive treatment from initial diagnosis. Mortality does not appear to increase with further increases in the antibody level.
Session topic: Platelet disorders 2
Keyword(s): Hemolytic anemia, Prognostic factor, Thrombotic thrombocytopenic purpura (TTP)
Type: Oral Presentation
Presentation during EHA21: On Sunday, June 12, 2016 from 09:00 - 09:15
Location: Room H4
Background
Acute Idiopathic Thrombotic Thrombocytopenic Purpura (TTP) is a life threatening disorder caused by inhibition of the Von Willebrand factor cleaving protein ADAMTS13 by acquired antibodies, usually of the IgG subclass. Its prevalence has been estimated at four to six cases per million per year and is characterized by hemolytic anemia, thrombocytopenia, fever, neurological symptoms and renal dysfunction. Untreated, mortality has been documented at 90% but even with treatment, it remains around 15-20%. Plasma Exchange (PEX) is the mainstay of treatment but immunosuppressive/immunomodulatory therapy is often also required. .Since January 2009, the United Kingdom TTP registry has been collecting information on all acute presentations of TTP across the country. In addition to testing for ADAMTS13 activity, confirmation of the presence of Anti-ADAMTS 13 IgG antibodies forms part of the initial work up.
Aims
A retrospective review to evaluate whether the presenting IgG ADAMTS13 antibody level can predict the likelihood of mortality in acute presentations of TTP.
Methods
360 acute events were identified from the registry involving 314 patients treated in 63 hospitals. Only acquired TTP was considered with all cases of congenital TTP excluded. Acute TTP was defined as ADAMTS13 protease activity below 10% (FRETS vWF-73 assay, NR: 60-120%) or between 10-20% with a detectable IgG ADAMTS13 antibody present. Acute events were then stratified into groups depending on the presenting IgG ADAMTS13 antibody level (0-29%, 30-59%, 60-89%, >90%).
Results
Of the events identified, 68% of those involved were female with a median age of 46 (range 5-90 years) and a median ADAMTS13 at diagnosis of <5% (range <5% – 57%). There was no statistically significant difference when considering these factors between the subgroups. 40 deaths were noted in total, accounting for 11.1% of all events. There was a statistically significant increase in mortality in patients with a presenting IgG ADAMTS13 antibody level above 30% compared to those with a level below 30% (p=0.0023). Subgroup analysis of those presenting with an IgG ADAMTS13 antibody level above 30% found no significant difference in mortality if the level was 30-59%, 60-89% or above 90% (p=0.83).
Conclusion
There is an increased risk of mortality seen in patients with acute TTP and a presenting IgG ADAMTS13 antibody level above 30%. Such patients should be considered for more aggressive treatment from initial diagnosis. Mortality does not appear to increase with further increases in the antibody level.
Session topic: Platelet disorders 2
Keyword(s): Hemolytic anemia, Prognostic factor, Thrombotic thrombocytopenic purpura (TTP)
Abstract: S827
Type: Oral Presentation
Presentation during EHA21: On Sunday, June 12, 2016 from 09:00 - 09:15
Location: Room H4
Background
Acute Idiopathic Thrombotic Thrombocytopenic Purpura (TTP) is a life threatening disorder caused by inhibition of the Von Willebrand factor cleaving protein ADAMTS13 by acquired antibodies, usually of the IgG subclass. Its prevalence has been estimated at four to six cases per million per year and is characterized by hemolytic anemia, thrombocytopenia, fever, neurological symptoms and renal dysfunction. Untreated, mortality has been documented at 90% but even with treatment, it remains around 15-20%. Plasma Exchange (PEX) is the mainstay of treatment but immunosuppressive/immunomodulatory therapy is often also required. .Since January 2009, the United Kingdom TTP registry has been collecting information on all acute presentations of TTP across the country. In addition to testing for ADAMTS13 activity, confirmation of the presence of Anti-ADAMTS 13 IgG antibodies forms part of the initial work up.
Aims
A retrospective review to evaluate whether the presenting IgG ADAMTS13 antibody level can predict the likelihood of mortality in acute presentations of TTP.
Methods
360 acute events were identified from the registry involving 314 patients treated in 63 hospitals. Only acquired TTP was considered with all cases of congenital TTP excluded. Acute TTP was defined as ADAMTS13 protease activity below 10% (FRETS vWF-73 assay, NR: 60-120%) or between 10-20% with a detectable IgG ADAMTS13 antibody present. Acute events were then stratified into groups depending on the presenting IgG ADAMTS13 antibody level (0-29%, 30-59%, 60-89%, >90%).
Results
Of the events identified, 68% of those involved were female with a median age of 46 (range 5-90 years) and a median ADAMTS13 at diagnosis of <5% (range <5% – 57%). There was no statistically significant difference when considering these factors between the subgroups. 40 deaths were noted in total, accounting for 11.1% of all events. There was a statistically significant increase in mortality in patients with a presenting IgG ADAMTS13 antibody level above 30% compared to those with a level below 30% (p=0.0023). Subgroup analysis of those presenting with an IgG ADAMTS13 antibody level above 30% found no significant difference in mortality if the level was 30-59%, 60-89% or above 90% (p=0.83).
Conclusion
There is an increased risk of mortality seen in patients with acute TTP and a presenting IgG ADAMTS13 antibody level above 30%. Such patients should be considered for more aggressive treatment from initial diagnosis. Mortality does not appear to increase with further increases in the antibody level.
Session topic: Platelet disorders 2
Keyword(s): Hemolytic anemia, Prognostic factor, Thrombotic thrombocytopenic purpura (TTP)
Type: Oral Presentation
Presentation during EHA21: On Sunday, June 12, 2016 from 09:00 - 09:15
Location: Room H4
Background
Acute Idiopathic Thrombotic Thrombocytopenic Purpura (TTP) is a life threatening disorder caused by inhibition of the Von Willebrand factor cleaving protein ADAMTS13 by acquired antibodies, usually of the IgG subclass. Its prevalence has been estimated at four to six cases per million per year and is characterized by hemolytic anemia, thrombocytopenia, fever, neurological symptoms and renal dysfunction. Untreated, mortality has been documented at 90% but even with treatment, it remains around 15-20%. Plasma Exchange (PEX) is the mainstay of treatment but immunosuppressive/immunomodulatory therapy is often also required. .Since January 2009, the United Kingdom TTP registry has been collecting information on all acute presentations of TTP across the country. In addition to testing for ADAMTS13 activity, confirmation of the presence of Anti-ADAMTS 13 IgG antibodies forms part of the initial work up.
Aims
A retrospective review to evaluate whether the presenting IgG ADAMTS13 antibody level can predict the likelihood of mortality in acute presentations of TTP.
Methods
360 acute events were identified from the registry involving 314 patients treated in 63 hospitals. Only acquired TTP was considered with all cases of congenital TTP excluded. Acute TTP was defined as ADAMTS13 protease activity below 10% (FRETS vWF-73 assay, NR: 60-120%) or between 10-20% with a detectable IgG ADAMTS13 antibody present. Acute events were then stratified into groups depending on the presenting IgG ADAMTS13 antibody level (0-29%, 30-59%, 60-89%, >90%).
Results
Of the events identified, 68% of those involved were female with a median age of 46 (range 5-90 years) and a median ADAMTS13 at diagnosis of <5% (range <5% – 57%). There was no statistically significant difference when considering these factors between the subgroups. 40 deaths were noted in total, accounting for 11.1% of all events. There was a statistically significant increase in mortality in patients with a presenting IgG ADAMTS13 antibody level above 30% compared to those with a level below 30% (p=0.0023). Subgroup analysis of those presenting with an IgG ADAMTS13 antibody level above 30% found no significant difference in mortality if the level was 30-59%, 60-89% or above 90% (p=0.83).
Conclusion
There is an increased risk of mortality seen in patients with acute TTP and a presenting IgG ADAMTS13 antibody level above 30%. Such patients should be considered for more aggressive treatment from initial diagnosis. Mortality does not appear to increase with further increases in the antibody level.
Session topic: Platelet disorders 2
Keyword(s): Hemolytic anemia, Prognostic factor, Thrombotic thrombocytopenic purpura (TTP)
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