Epstein-Barr virus-associated T-lymphoproliferative disease with hemophagocytic syndrome, followed by fatal intestinal B lymphoma in a young adult female with WHIM syndrome. Warts, hypogammaglobulinemia, infections, and myelokathexis

Ann Hematol. 2002 Aug;81(8):470-3. doi: 10.1007/s00277-002-0489-9. Epub 2002 Jul 20.


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.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Fatal Outcome
  • Female
  • Herpesvirus 4, Human
  • Histiocytosis, Non-Langerhans-Cell / blood
  • Histiocytosis, Non-Langerhans-Cell / complications*
  • Humans
  • Intestinal Neoplasms / blood
  • Intestinal Neoplasms / pathology*
  • Lymphoma, B-Cell / blood
  • Lymphoma, B-Cell / pathology*
  • Lymphoproliferative Disorders / blood
  • Lymphoproliferative Disorders / complications*
  • Lymphoproliferative Disorders / virology
  • Neutrophils / pathology*
  • T-Lymphocytes / immunology
  • T-Lymphocytes / pathology