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Abstract: PO2265

Chronic Neurological Impairment in Patients with Thrombotic Thrombocytopenic Purpura: Preliminary Findings in a Comprehensive MRI Protocol

Session Information

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
SequenceNumber of Participants with at Least One AbnormalityExample of Abnormality
T2-Weighted (FLAIR)10White matter hyperintensity
T1-Weighted7Atrophy of white matter or gray matter
Angiography5Thrombus
Susceptibility-Weighted3Microbleed
Diffusion-Weighted1Siderosis

Funding

  • Private Foundation Support