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Delayed catastrophic antiphospholipid syndrome after massive cerebral infarction
Wei Hsi Chen1, Hung Sheng Lin, Yi Fen Kao
1Neurocritical Care Unit, Department of Neurology, Chang Gung Memorial Hospital-Kaohsiung Medical Center, and School of Medicine, Chang Gung University, 123 Ta Pei Road, Niao Sung Hsiang, Kaohsiung 833, Taiwan. e49130@ms14.hinet.net
Neurocritical Care
|June 15, 2007
Summary
Catastrophic antiphospholipid syndrome (CAPS) can occur even with normal coagulation tests. Rapid diagnosis and treatment, including plasmapheresis, are crucial for improving outcomes in patients with this life-threatening condition.
Area of Science:
- Rheumatology
- Hematology
- Neurology
Background:
- Catastrophic antiphospholipid syndrome (CAPS) is a rare, severe systemic autoimmune disease.
- It is characterized by widespread thromboses and organ damage, requiring urgent intervention.
- Prompt diagnosis is essential for effective management and improved patient prognosis.
Observation:
- A patient with Sjogren syndrome developed CAPS, presenting with unconsciousness following cerebral ischemia.
- Initial laboratory tests, including thrombocyte count and coagulation times, were within normal limits.
- Elevated anti-beta(2)-glycoprotein I antibody levels were detected, indicating antiphospholipid antibodies.
Findings:
- The case highlights that normal hemostatic parameters do not rule out CAPS.
- Plasmapheresis was administered, leading to a rapid reversal of the patient's consciousness and coagulation function.
- High anti-beta(2)-glycoprotein I antibody levels are a key diagnostic marker for CAPS.
Implications:
- Clinicians should consider CAPS in patients with unexplained thrombotic events, even with normal coagulation profiles.
- Early recognition and aggressive treatment, such as plasmapheresis, can significantly improve outcomes in CAPS.
- This case underscores the importance of antibody testing in diagnosing antiphospholipid syndrome variants.
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