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Epstein–Barr virus-associated haemophagocytic lympho-histiocytosis after stem cell transplantation

Bone Marrow Transplantation volume 38, pages 709–710 (2006)Cite this article

Epstein–Barr virus (EBV) is one of the most ubiquitous infectious viruses with >90% of the world's population infected with this human herpes virus.1, 2 In the immunocompetent host, EBV latently infects B cells without any pathological disorders, but a significant role of EBV has also been suggested in the pathogenesis of a number of diseases of (non-)hematological origin.3, 4, 5, 6 In immune-incompetent patients, such as patients after stem cell and solid organ transplantation, post transplant lymphoproliferative disease (PTLD: median onset 3 months, range 1 week to 9 years) is a well-known complication associated with EBV reactivation. The incidence ranges from 1 to 20% in haematopoietic stem cell transplantation (SCT) recipients, with markedly increased incidences in patients receiving T-cell depleted grafts.3, 4 Haemophagocytic lympho-histiocytosis (HLH) is a very rare complication after SCT.6, 7 Here, we report a unique case of a patient who developed a HLH syndrome with high EBV loads detected in the NK-cell compartment of the peripheral blood.

The increase of plasmatic EBV load after anti-CD20 therapy might be due to massive outflow of EBV from the lysed B cells. However, because of the clinical deterioration we suspected other cell fractions to be EBV positive. Using the CliniMACSR cell selection system, CD19+, CD3+ and CD16/56+ (NK) cells (95% purified) were positively selected. We measured viral loads on those cell fractions (copies/105 cells) by EBV-DNA PCR. A very high intracellular viral load in the NK cells, and a lower viral load in the T cells was found (see Figure 1). As we suspected transmission of EBV owing to infected NK cells of the donor, plasmatic and intracellular load in T-, NK- and B cells of the donor was measured. The viral loads in the various cell fractions of the haplo-donor were 16 000 copies/105 NK cells, 200 copies/105 T cells, 500 copies/105 B cells and plasmatic loads were negative.

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Authors and Affiliations

  1. Department of Clinical Immunology and Bone Marrow Transplantation, Wilhelmina Children Hospital, UMC Utrecht, The Netherlands

    J J Boelens & N M Wulffraat

  2. Department of Immunology and Pediatric Rheumatology, Fellow Clinical Immunology, National Children's Hospital Dr Carlos Sáenz Herrera, San José, Costa Rica

    G Lazo

  3. Department of Immunology, Wilhelmina Children's Hospital, UMC Utrecht, The Netherlands

    J F Gaiser

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  1. J J Boelens
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  2. G Lazo
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  4. N M Wulffraat
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Correspondence to J J Boelens.

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Boelens, J., Lazo, G., Gaiser, J. et al. Epstein–Barr virus-associated haemophagocytic lympho-histiocytosis after stem cell transplantation. Bone Marrow Transplant 38, 709–710 (2006). https://doi.org/10.1038/sj.bmt.1705517

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