Late-Onset Fatal Epstein-Barr Virus—Associated Hemophagocytic Syndrome Following Cord Blood Cell Transplantation for Adult Acute Lymphoblastic Leukemia


A 43-year-old Japanese woman underwent unrelated cord blood transplantation (CBT) during remission for acute lymphoblastic leukemia with t(4;11)(q21;q23). Tacrolimus was given for prophylaxis of graft-versus-host disease. The posttransplantation clinical course was mostly uneventful, and the leukemia remained in remission. Fourteen months after CBT, the patient developed pancytopenia and hepatic dysfunction with persistent high-grade fever. The bone marrow was hypocellular with increased numbers of macrophages and hemophagocytes. The numbers of Epstein-Barr virus (EBV) copies in peripheral blood samples were remarkably high. Although the patient showed complete donor-type hematopoiesis, the titer of viral capsid antigen immunoglobulin G was low, and the results of a test for EBV nuclear antigen were negative. There was no clinical response to the reduction of immunosuppressive therapy or to the administration of high-dose methylprednisolone, human immunoglobulin, or acyclovir.The patient died 466 days after CBT of massive gastrointestinal hemorrhage due to bone marrow and hepatic failures. This case demonstrates that fatal EBV-associated hemophagocytic syndrome (HPS) can occur more than 1 year after CBT. This report is the first of a case of late-onset EBV-associated HPS following CBT.

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Correspondence to Yoshinari Kawabata or Makoto Hirokawa or Yoshinobu Saitoh or Shigeki Kosugi or Tomoko Yoshioka or Masumi Fujishima or Naohito Fujishima or Yoshihiro Kameoka or Hirobumi Saitoh or Masaaki Kume or Naoto Takahashi or Ken-ichi Sawada.

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Kawabata, Y., Hirokawa, M., Saitoh, Y. et al. Late-Onset Fatal Epstein-Barr Virus—Associated Hemophagocytic Syndrome Following Cord Blood Cell Transplantation for Adult Acute Lymphoblastic Leukemia. Int J Hematol 84, 445–448 (2006).

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Key words

  • PTLD
  • EBV
  • HPS
  • CBT
  • Late onset