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Rare disease
Overwhelming primary Epstein–Barr virus infection requiring corticosteroid treatment
  1. Marije Gordinou de Gouberville1,2,
  2. Martien Janssen1,
  3. Anita Schrander-van der Meer1,
  4. Matthijs Eefting3,
  5. Nathalie Delfos1,
  6. Cees van Nieuwkoop4
  1. 1Department of Internal medicine, Rijnland Hospital, Leiderdorp, Netherlands
  2. 2Surgical Department, Ligula Hospital, Mtwara, Tanzania
  3. 3Department of Haematology, Leiden University Medical Centre, Leiden, Netherlands
  4. 4Department of Internal medicine, Hagaziekenhuis, Den Haag, Netherlands
  1. Correspondence to Miss Marije Gordinou de Gouberville, mc.gordinou{at}


A 20-year-old woman presented with a 2-week history of fever and malaise. Physical examination was unremarkable. Viral infection was suspected and Epstein–Barr virus serology confirmed acute infectious mononucleosis. During admission, she gradually developed pancytopenia and liver enzyme abnormalities. The patient clinically deteriorated with persisting fever, orthostatic hypotension and hepatosplenomegaly. Bone marrow examination showed haemophagocytic lymphohistiocytosis (HLH). Treatment with high-dose corticosteroids was started and patient recovered quickly. Ferritin decreased immediately, fever resolved within 3 days, viral clearance was reached within 3 weeks. Steroid therapy was gradually tapered off in three months. The Histiocyte Society recommends immunochemotherapy with steroids, etoposide and cyclosporine. Potential side effects of etoposide are severe bone marrow depression and leukaemia. Our patient survived on corticosteroids alone. Early recognition of HLH and prompt treatment are of utmost importance for survival. Treatment with steroids alone can be life-saving.

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  • Competing interests None.

  • Patient consent Not obtained.

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