Abstract
A rare association of Epstein-Barr virus-associated T- and B-lymphoproliferative disease (EBV+ T- and EBV+ B-LPD) in a patient with WHIM (warts, hypogammaglobulinemia, infections, and myelokathexis) syndrome is reported. A 26-year-old Japanese female, who had been treated for WHIM syndrome since early childhood, developed hemophagocytic syndrome associated with EBV+ T-LPD at the lymph nodes and spleen. The disease rapidly resolved in response to prednisolone therapy. However, 6 weeks later, fatal EBV+ B lymphoma unresponsive to chemotherapy occurred in the intestine and other organs. Caution must be exercised that the patient with WHIM syndrome may be at risk for EBV-LPD.
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Imashuku, S., Miyagawa, A., Chiyonobu, T. et al. Epstein-Barr virus-associated T-lymphoproliferative disease with hemophagocytic syndrome, followed by fatal intestinal B lymphoma in a young adult female with WHIM syndrome. Ann Hematol 81, 470–473 (2002). https://doi.org/10.1007/s00277-002-0489-9
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DOI: https://doi.org/10.1007/s00277-002-0489-9