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Coma in thrombotic thrombocytopenic purpura
F J de Jong1, P A W Te Boekhorst, D W J Dippel
1Department of Neurology, Erasmus Medical Center, Rotterdam, The Netherlands. f.j.dejong@erasmusmc.nl
BMJ Case Reports
|July 17, 2012
Summary
Thrombotic thrombocytopenic purpura (TTP) can cause coma and neurological issues. This case highlights TTP diagnosis and recovery despite normal ADAMTS13 levels, emphasizing prompt treatment for better outcomes.
Area of Science:
- Neurology
- Hematology
- Critical Care Medicine
Background:
- Thrombotic thrombocytopenic purpura (TTP) is a rare hematologic disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ damage.
- Neurological symptoms are common in TTP, ranging from headache to coma, posing diagnostic challenges.
Observation:
- A 55-year-old male presented with abdominal pain and rapid neurological decline into coma.
- Initial presentation mimicked hypertensive crisis, but antihypertensive treatment provided no improvement.
- Brain imaging ruled out hypertensive leucoencephalopathy.
Findings:
- Idiopathic TTP was diagnosed despite normal disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13 (ADAMTS13) activity.
- The patient showed gradual improvement following plasma exchange, corticosteroids, and vincristine therapy.
- Neurological deficits resolved, leaving mild cognitive deficits and polyneuropathy.
Implications:
- This case underscores the importance of considering TTP in patients with unexplained neurological deterioration, even with normal ADAMTS13 levels.
- Aggressive treatment including plasma exchange is crucial for TTP management and improving patient prognosis.
- Early diagnosis and intervention can mitigate severe organ damage and improve long-term neurological recovery in TTP patients.
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