Contributions
Abstract: PB2108
Type: Publication Only
Background
Whilst immune mediated Thrombotic Thrombocytopenic Purpura (TTP) has classically been suspected by the presence of a pentad of symptoms (microangiopathic haemolytic anaemia, fever, disturbed neurological function, renal failure, thrombocytopenia), the limitations of this have long been recognized and a wide variety of symptoms are seen on initial presentation.
Aims
A retrospective review of the significance of specific symptoms and their duration on mortality.
Methods
A retrospective review of all consecutive admissions to a single tertiary center between 2009 and 2015. Only patients who required plasma exchange were included. Patients’ symptoms and their duration were reviewed in addition to presenting anti-ADAMTS13 IgG antibody levels and ADAMTS13 antigen levels, both of which have previously been found to have prognostic significance.
Results
106 patients (68% female) were included with a median age of 48. 58% were Caucasian and 19.8% Afro-Caribbean. The mortality rate was 7.4% (n=8). 47% of patients had neurological symptoms on presentation, 24% reported a bleeding history and 12% a recent infection. The most common presenting symptoms were headache (27.4%), bleeding (24%) spontaneous bruising/petechial rashes (19.8%), speech disturbances (encompassing expressive/receptive dysphasia, aphasia, dysarthia and slurred speech, 19.8%) and TIA or stroke like symptoms encompassing hemiplegia or facial weakness/droop (16%). The highest rates of mortality were seen in patients who experienced loss of consciousness (mortality 33.3%), abdominal pain (mortality 22.2%) and heavy bleeding (mortality 16.7%). The anti-ADAMTS13 IgG level was not however significantly higher in these symptoms when compared to others (table 1) suggesting microangiopathic thrombosis location plays an important role in TTP prognosis.
Symptom (n) | Mortality | Antibody (NR: <6%) | Antigen (NR: 74-134%) |
LOC (6) | 33.3% | 44% | 2.9% |
Abdominal pain (9) | 22.2% | 50% | 3.3% |
Heavy bleeding (18) | 16.7% | 28% | 2.9% |
Headache (29) | 3% | 44% | 4.5% |
Speech Disturbance (21) | 4.8% | 48% | 4.2% |
Tia/stroke symptoms (17) | 5.9% | 42% | 4.8% |
Pyrexia (16) | 12.5% | 32% | 5.7% |
Lethargy (15) | 13.3% | 58% | 7% |
Spontaneous Bruising (15) | 6.7% | 56% | 6.4% |
Confusion (10) | 0% | 48% | 4.2% |
SOB (10) | 10% | 45% | 4.1% |
Conclusion
Whilst there is little difference in the anti-ADAMTS13 IgG antibody and ADAMTS13 levels seen with difference symptoms, there is a wide disparity in terms of mortality suggesting the effect of microangiopathic thrombosis differs by location. Abdominal pain, not previously recognized as a significant symptom in TTP, seems to be a poor prognostic indicator although this should be interpreted with caution given the sample size.
Session topic: 32. Platelets disorders
Keyword(s): Thrombotic thrombocytopenic purpura (TTP), Thrombotic microangiopathy, Prognostic factor, ADAMTS13
Abstract: PB2108
Type: Publication Only
Background
Whilst immune mediated Thrombotic Thrombocytopenic Purpura (TTP) has classically been suspected by the presence of a pentad of symptoms (microangiopathic haemolytic anaemia, fever, disturbed neurological function, renal failure, thrombocytopenia), the limitations of this have long been recognized and a wide variety of symptoms are seen on initial presentation.
Aims
A retrospective review of the significance of specific symptoms and their duration on mortality.
Methods
A retrospective review of all consecutive admissions to a single tertiary center between 2009 and 2015. Only patients who required plasma exchange were included. Patients’ symptoms and their duration were reviewed in addition to presenting anti-ADAMTS13 IgG antibody levels and ADAMTS13 antigen levels, both of which have previously been found to have prognostic significance.
Results
106 patients (68% female) were included with a median age of 48. 58% were Caucasian and 19.8% Afro-Caribbean. The mortality rate was 7.4% (n=8). 47% of patients had neurological symptoms on presentation, 24% reported a bleeding history and 12% a recent infection. The most common presenting symptoms were headache (27.4%), bleeding (24%) spontaneous bruising/petechial rashes (19.8%), speech disturbances (encompassing expressive/receptive dysphasia, aphasia, dysarthia and slurred speech, 19.8%) and TIA or stroke like symptoms encompassing hemiplegia or facial weakness/droop (16%). The highest rates of mortality were seen in patients who experienced loss of consciousness (mortality 33.3%), abdominal pain (mortality 22.2%) and heavy bleeding (mortality 16.7%). The anti-ADAMTS13 IgG level was not however significantly higher in these symptoms when compared to others (table 1) suggesting microangiopathic thrombosis location plays an important role in TTP prognosis.
Symptom (n) | Mortality | Antibody (NR: <6%) | Antigen (NR: 74-134%) |
LOC (6) | 33.3% | 44% | 2.9% |
Abdominal pain (9) | 22.2% | 50% | 3.3% |
Heavy bleeding (18) | 16.7% | 28% | 2.9% |
Headache (29) | 3% | 44% | 4.5% |
Speech Disturbance (21) | 4.8% | 48% | 4.2% |
Tia/stroke symptoms (17) | 5.9% | 42% | 4.8% |
Pyrexia (16) | 12.5% | 32% | 5.7% |
Lethargy (15) | 13.3% | 58% | 7% |
Spontaneous Bruising (15) | 6.7% | 56% | 6.4% |
Confusion (10) | 0% | 48% | 4.2% |
SOB (10) | 10% | 45% | 4.1% |
Conclusion
Whilst there is little difference in the anti-ADAMTS13 IgG antibody and ADAMTS13 levels seen with difference symptoms, there is a wide disparity in terms of mortality suggesting the effect of microangiopathic thrombosis differs by location. Abdominal pain, not previously recognized as a significant symptom in TTP, seems to be a poor prognostic indicator although this should be interpreted with caution given the sample size.
Session topic: 32. Platelets disorders
Keyword(s): Thrombotic thrombocytopenic purpura (TTP), Thrombotic microangiopathy, Prognostic factor, ADAMTS13