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

Bone Marrow Transplantation volume 27, pages 883–886 (2001)Cite this article

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. Bone Marrow Transplantation (2001) 27, 883–886.

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Acknowledgements

We are indebted to the Tokai Cord Blood Bank for supplying the cord blood stem cells. We are also grateful to Dr Hiroshi Kimura (Nagoya University School of Medicine, Nagoya, Japan) for providing the BALF5 gene construct. This work was supported in part by a Grant-in-Aid for Scientific Research from the Ministry of Education, Science, Sports and Culture, Japan.

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Authors and Affiliations

  1. Department of Pediatric Oncology, Institute of Development, Aging and Cancer, Tohoku University, Sendai, Japan

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

  2. Division of Blood Transfusion, Tohoku University School of Medicine, Sendai, Japan

    M Minegishi

  3. Department of Pediatrics, Sendai National Hospital, Sendai, Japan

    T Sato

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  1. M Minegishi
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  2. Y Ohashi
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  3. S Kumaki
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Minegishi, M., Ohashi, Y., Kumaki, S. et al. Successful umbilical cord blood transplantation from an unrelated donor for a patient with Epstein–Barr virus-associated hemophagocytic lymphohistiocytosis. Bone Marrow Transplant 27, 883–886 (2001). https://doi.org/10.1038/sj.bmt.1702999

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