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Minimal T-cell requirements for triggering haemophagocytosis associated with Epstein–Barr virus-driven B-cell proliferation: a clinical case study
  1. Thomas Daikeler1,
  2. Alexandar Tzankov2,
  3. Gideon Hoenger3,
  4. Olivier Gasser3,
  5. Alan Tyndall1,
  6. Alois Gratwohl4,
  7. Christoph Hess3,5
  1. 1Division of Rheumatology, University Hospital Basel, Basel, Switzerland
  2. 2Department of Pathology, University Hospital Basel, Basel, Switzerland
  3. 3Immunobiology Laboratory, Department of Biomedicine, University of Basel, Basel, Switzerland
  4. 4Division of Hematology, University Hospital Basel, Basel, Switzerland
  5. 5Medical Outpatient Department, University Hospital, Basel, Switzerland
  1. Correspondence to Professor Christoph Hess, University Hospital Basel, CH-4031 Basel, Switzerland; chess{at}uhbs.ch

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The pathophysiology of Epstein–Barr virus (EBV)-associated haemophagocytosis remains poorly understood.1 In EBV-related haemophagocytic lymphohistiocytosis, EBV-infected CD8 T cells and natural killer cells are thought to trigger haemophagocytosis and the associated ‘cytokine-storm’/systemic inflammatory response syndrome (SIRS) directly.2,,4

By contrast, in the context of uncontrolled proliferation of EBV-infected B cells, hyperactively responding EBV-specific T cells are assumed to mediate haemophagocytosis/SIRS.5 Both the quantity and quality of such deregulated EBV-specific T-cell reactivity remain undefined. Unique insight into basic aspects of the postulated T-cell requirements necessary to trigger haemophagocytosis was provided by the case of a 37-year-old woman with mixed connective tissue disease (ribonucleoprotein-Ab positive, severe pulmonary arterial hypertension, polyarthritis, pericarditis and oesophageal sclerosis). Immunosuppression with azathioprine and ciclosporin, as well as a 3-month trial of oral cyclophosphamide due to progressing pulmonary arterial hypertension was ineffective, yet worsened pre-existing lymphopenia. Based on the severe and cyclophosphamide-resistant course of the disease the indication for haematopoietic stem cell transplantation (HSCT) was established. …

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