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J Neurol Surg A Cent Eur Neurosurg 2024; 85(06): 625-632
DOI: 10.1055/a-2271-8772
Case Report

Spontaneous Extradural Hematoma in a Sickle Cell Anemia Patient with Hyperinflammation and Thrombotic Microangiopathy Successfully Treated with Eculizumab: A Case Report and Review of the Literature

Michael Itak Ita 
1   Department of Neurological Surgery, Aalborg University Hospital, Aalborg, Denmark
,
Pia Olesen
1   Department of Neurological Surgery, Aalborg University Hospital, Aalborg, Denmark
,
Maria Rosing
1   Department of Neurological Surgery, Aalborg University Hospital, Aalborg, Denmark
,
Morten Mørk
2   Department of Clinical Biochemistry, Aalborg University Hospital, Aalborg, Denmark
,
Halldór Bjarki Einarsson
1   Department of Neurological Surgery, Aalborg University Hospital, Aalborg, Denmark
,
Jens Jakob Riis
1   Department of Neurological Surgery, Aalborg University Hospital, Aalborg, Denmark
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Abstract

Background The event of extradural hematoma in the absence of head trauma is a rare central nervous system complication of sickle cell disease. We report here a case of spontaneous extradural hematoma in a patient being treated for sickle cell vasoocclusive crisis complicated by hyperinflammation and thrombotic microangiopathy. The significance of inflammation as an integral component of the pathomechanism of vasoocclusive crisis in patients with sickle cell disease and the role of heme in activating the complement system's alternative pathway are highlighted in this case report.

Case Presentation A teenage patient with sickle cell disease developed a spontaneous right parietal extradural hematoma while receiving treatment for sickle cell vasoocclusive crisis. The concurrent events of hyperinflammation, disseminated intravascular coagulation, hyperhemolysis syndrome, thrombotic microangiopathy, and refractory postoperative bleeding complicated this patient's clinical course after surgical evacuation of extradural hematoma. This patient was subsequently treated with eculizumab and improved in the days following.

Conclusion Treatment with the anti-C5 monoclonal antibody eculizumab, which targets and inhibits terminal complement system activation, reversed the deleterious cascade of events in this patient with sickle cell disease.

Keywords

spontaneous extradural hematoma - hyperinflammation - thrombotic microangiopathy - sickle cell vasoocclusive crisis - eculizumab

Supplementary Material



Publication History

Received: 23 November 2023

Accepted: 14 February 2024

Accepted Manuscript online:
20 February 2024

Article published online:
03 July 2024

© 2024. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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