Abstract: PO2265
Chronic Neurological Impairment in Patients with Thrombotic Thrombocytopenic Purpura: Preliminary Findings in a Comprehensive MRI Protocol
Session Information
- Pathology and Lab Medicine: Clinical
October 22, 2020 | Location: On-Demand
Abstract Time: 10:00 AM - 12:00 PM
Category: Pathology and Lab Medicine
- 1602 Pathology and Lab Medicine: Clinical
Authors
- Hamilton, Jeff, Western University, London, Ontario, Canada
- Jurkiewicz, Michael T., Western University, London, Ontario, Canada
- Thiessen, Jonathan D., Western University, London, Ontario, Canada
- Huang, Shih-Han S., Western University, London, Ontario, Canada
Background
Thrombotic thrombocytopenic purpura (TTP) is a life-threating blood disorder characterized by insufficient activity in ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motif, member 13). This protein prevents blood clotting, so in TTP there is spontaneous clotting throughout the microvasculature. Treatment includes plasma exchange therapy and immunosuppressants to improve fever, thrombocytopenia, and kidney failure. However, neurological changes, such as seizures and confusion, persist. There is limited research on TTP on the brain but it is known that TTP presents similar pathology to stroke. This study aims to better understand the long-term impact of TTP on the brain using a comprehensive magnetic resonance imaging (MRI) protocol and cognitive testing.
Methods
13 patients (5 male, mean age 44.5) in hematological remission were recruited. Participants had a 65-minute MRI scan (Siemens mMR Biograph 3T) based on best-practice guidelines for imaging stroke. The protocol included five qualitative acqusitions and three quantitative acquisitions. Participants also completed a 40-minute cognitive test (Cambridge Brain Sciences) to assess cognitive impairment.
Results
Table 1 summarizes the findings across qualitative acquisitions. The most salient findings were the white matter hyperintensities seen in the T2 FLAIR in Image 1. 12 participants completed the cognitive testing and there is evidence of cognitive impairment.
Conclusion
Patients will be scanned again at six and twelve months. Age-matched healthy controls will be recruited before drawing conclusions on quantitative metrics, namely the amount of white matter. These early results are promising for better understanding the implications of TTP on the brain.
Summary of qualitative findings
| Sequence | Number of Participants with at Least One Abnormality | Example of Abnormality |
| T2-Weighted (FLAIR) | 10 | White matter hyperintensity |
| T1-Weighted | 7 | Atrophy of white matter or gray matter |
| Angiography | 5 | Thrombus |
| Susceptibility-Weighted | 3 | Microbleed |
| Diffusion-Weighted | 1 | Siderosis |
Funding
- Private Foundation Support