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Published in: Neurocritical Care 1/2018

01-02-2018 | Practical Pearl

Probable Catastrophic Antiphospholipid Syndrome with Intracerebral Hemorrhage Secondary to Epstein–Barr Viral Infection

Authors: Mark P. Plummer, Adam M. H. Young, Ronan O’Leary, Maxwell S. Damian, Andrea Lavinio

Published in: Neurocritical Care | Issue 1/2018

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Abstract

Background

Catastrophic antiphospholipid syndrome (CAPS) is a rare, severe variant of antiphospholipid syndrome with a high mortality rate. We report a unique case of CAPS secondary to Epstein–Barr viral (EBV) infection complicated by pulmonary and intracerebral hemorrhage. A review of the CAPS literature relevant to intensive care practice is used to outline a rational approach to diagnosis and management.

Methods

All data are from a single patient admitted to the Neurosciences Critical Care Unit in Addenbrooke’s Hospital, Cambridge, in March 2016. Medline, Web of Science, PubMed, and the Cochrane Library were searched through September 2016 without restrictions for cases of CAPS, management of CAPS in the intensive care unit, and hemorrhage complicating CAPS. The patient gave express written consent to access and publish these data.

Results

This is only the second reported case of probable CAPS secondary to EBV infection. Furthermore, pulmonary and intracerebral hemorrhage is rare manifestations of this multisystem prothrombotic state which provided unique challenges to the management.

Conclusions

While rare, CAPS should be considered in any patient presenting with rapidly progressive multiorgan failure, evidence of thrombotic microangiopathy, and antiphospholipid antibodies. A high index of suspicion is required as early, aggressive, multimodal treatment with anticoagulation, and immunosuppression improves outcomes.
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Metadata
Title
Probable Catastrophic Antiphospholipid Syndrome with Intracerebral Hemorrhage Secondary to Epstein–Barr Viral Infection
Authors
Mark P. Plummer
Adam M. H. Young
Ronan O’Leary
Maxwell S. Damian
Andrea Lavinio
Publication date
01-02-2018
Publisher
Springer US
Published in
Neurocritical Care / Issue 1/2018
Print ISSN: 1541-6933
Electronic ISSN: 1556-0961
DOI
https://doi.org/10.1007/s12028-017-0392-9

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