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Hyperacute GVHD and emergence of peripheral CD3+CD56+ T cells and activated natural killer cells are useful markers for early diagnosis of post-transplant hemophagocytic syndrome

Bone Marrow Transplantation volume 35, pages 415–417 (2005)Cite this article

Hemophagocytic syndrome (HPS) is a cytokine-related disorder characterized by sustained high-grade fever, pancytopenia, hepatomegaly, coagulopathy and hemophagocytosis in the marrow, spleen, or lymph nodes.1 The development of HPS is related to underlying immune dysregulation and inappropriate activation of macrophages. In adults, viral, bacterial, and fungal infections and/or malignant lymphoma are associated with HPS.1

HPS is a rare complication following stem-cell transplantation (SCT), and occasional case reports only have been published.2,3 Besides infection and hematologic malignancies, immune reactions particularly hypercytokinemia following SCT have been associated with HPS. Although some patients with early-onset HPS respond to corticosteroids, HPS is often fatal. At present, its clinical characteristics remain unknown. We report a patient who developed early-onset HPS after allogeneic SCT. A 30-year-old man with chemorefractory acute myeloid leukemia was referred to our hospital for allogeneic SCT in November 2003. After cytoreduction using cytarabine, he underwent peripheral blood SCT from his one-locus-mismatched sister in January 2004. The preparative regimen consisted of busulfan 4 mg/kg/day for 4 days and cyclophosphamide 60 mg/kg for 2 days. Graft-versus-host disease (GVHD) prophylaxis was tacrolimus 0.03 mg/kg/day. Methotrexate was omitted from GVHD prophylaxis to enhance a graft-versus-leukemia effect. The numbers of infused CD34+ cells and CD3+ cells were 2.6 × 106/kg and 3.2 × 108/kg, respectively.

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Authors and Affiliations

  1. Hematopoietic Stem Cell Transplantation Unit, The National Cancer Center Hospital, Tokyo, Japan

    Y Kishi, M Kami, N Murashige, G Fujisaki, S Kusumoto, S-I Mori, Y Takaue & R Tanosaki

  2. Program in Molecular Therapeutics, Department of Surgery, Duke University Medical Center, Durham, NC, USA

    Y Tanaka

  3. Department of Hematology and Oncology, University of Tokyo, Tokyo, Japan

    K Haraguchi

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  1. Y Kishi
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Kishi, Y., Kami, M., Murashige, N. et al. Hyperacute GVHD and emergence of peripheral CD3+CD56+ T cells and activated natural killer cells are useful markers for early diagnosis of post-transplant hemophagocytic syndrome. Bone Marrow Transplant 35, 415–417 (2005). https://doi.org/10.1038/sj.bmt.1704771

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