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Rare case of catastrophic antiphospholipid syndrome with spontaneous intracranial haemorrhage
  1. Anju Adhikari1,
  2. Mohammad Muhsin Chisti2,
  3. Sanjog Bastola1 and
  4. Ojbindra KC1
  1. 1 Internal Medicine, Oakland University William Beaumont School of Medicine, Royal Oak, Michigan, USA
  2. 2 Hematology and Medical Oncology, Oakland University William Beaumont School of Medicine, Royal Oak, Michigan, USA
  1. Correspondence to Dr Anju Adhikari, anju.adhikari{at}


Catastrophic antiphospholipid syndrome (CAPS) is a rare but severe form of antiphospholipid syndrome (APS). The syndrome manifests itself as a rapidly progressive multiorgan failure that is believed to be caused by widespread micro-thrombosis. Seldom does bleeding comanifest with thrombosis. We present a patient with APS who presented with nausea, vomiting and fatigue, and rapidly progressed into multiorgan failure before being diagnosed with CAPS. The clinical course was complicated by an atraumatic intracranial haemorrhage which demanded discontinuation of anticoagulation. The patient was treated with high dose steroid, intravenous immunoglobulin, followed by weekly rituximab infusion. Although the trigger for CAPS was not obvious during her hospital stay, she was diagnosed with acute cytomegalovirus (CMV) infection soon after discharge. In this case report, we explore the differential diagnoses of CAPS, investigate the possibility of CMV infection as a potential trigger, present the therapeutic challenges of anticoagulation and discuss the emerging use of rituximab.

  • immunology
  • haematology (incl blood transfusion)
  • haematology (drugs and medicines)
  • biological agents
  • rheumatology

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  • Contributors AA is the primary author. She conceptualised the case, performed literature review, wrote and finalised the manuscript. MMC is the guarantor who contributed in editing the manuscript and accepts full responsibility of the work. SB and OKC are contributors to the case reports who assisted with the literature review and editing the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Obtained.