Successful umbilical cord blood transplantation from an unrelated donor for a patient with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis

M. Minegishi, Y. Ohashi, S. Kumaki, Y. Sasahara, T. Hayashi, H. Asada, T. Okuyama, I. Hakozaki, T. Sato, S. Tsuchiya

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20 Citations (Scopus)

Abstract

We report a case of a 5-year-old girl with EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH) who underwent cord blood (CB) stem cell transplantation (CBSCT) from an unrelated donor. The patient presented with persistent high-grade fever and hepatosplenomegaly. Because the disease was refractory to immunochemotherapy according to the HLH94 protocol, she received 2.0 × 107 CB nucleated cells/kg body weight (BW) after conditioning with BU/CY/etoposide. No acute GVHD developed, using FK506 for prophylaxis. The neutrophil count reached >0.5 × 109/l by day 21 and the platelet count reached >50 × 109/l by day 84. The patient recovered well with sequelae of neurological deficits more than 10 months after receiving CBSCT, without showing evidence of HLH or chronic GVHD. Real-time PCR proved applicable for estimation of the EBV load in PBMC of the patient. We conclude that CBSCT may be indicated for some cases of refractory EBV-HLH, who have no HLA-matched siblings and are therefore dependent on unrelated marrow donors.

Original languageEnglish
Pages (from-to)883-886
Number of pages4
JournalBone Marrow Transplantation
Volume27
Issue number8
DOIs
Publication statusPublished - 2001 Jun 12

Keywords

  • Allogeneic transplantation
  • EBV
  • Hemophagocytic lymphohistiocytosis
  • Real-time PCR
  • Umbilical cord blood stem cell

ASJC Scopus subject areas

  • Hematology
  • Transplantation

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