Cord Blood Transplantation Following Reduced-Intensity Conditioning for Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis during Systemic Lupus Erythematosus Treatment

Takuro Kuriyama, Noriaki Kawano, Kiyoshi Yamashita, Ikuo Kikuchi

    Research output: Contribution to journalArticle

    2 Citations (Scopus)

    Abstract

    Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a serious disorder in which monoclonal growth of T cells infected with EBV and macrophage activation cause pancytopenia, high fever and acute liver failure. Patients with chemotherapy- or immunosuppression-resistant EBV-HLH require allogeneic hematopoietic stem cell transplantation (allo-HSCT), but patients who have no sibling donors may not have time to wait for an unrelated donor. In pediatric patients, there are some reports on allogeneic cord blood transplantation (allo-CBT) for the treatment of EBV-HLH; however, in adult patients, reports of allo-CBT for EBV-HLH are quite limited. The present case of a 20-year-old woman with chemotherapy-resistant EBV-HLH and systemic lupus erythematosus (SLE) who underwent allo-CBT following reduced-intensity conditioning (RIC-CBT). She achieved and maintained a complete donor type and the EBV-DNA load, and the titers of anti-double stranded DNA and antinuclear antibodies became negative. It is therefore considered that RIC-CBT is an effective treatment option for adult onset HLH. However, because the effectiveness of allo-HSCT for SLE remains unclear and transplant-related mortality is high, it is recommended that allo-HSCT for SLE is restricted to patients concomitant with oncohematological disease as with our present case.

    Original languageEnglish
    Pages (from-to)126-129
    Number of pages4
    JournalJournal of clinical and experimental hematopathology : JCEH
    Volume56
    Issue number2
    DOIs
    Publication statusPublished - Jan 1 2016

    Fingerprint

    Hemophagocytic Lymphohistiocytosis
    Human Herpesvirus 4
    Fetal Blood
    Systemic Lupus Erythematosus
    Transplantation
    Hematopoietic Stem Cell Transplantation
    Therapeutics
    Tissue Donors
    Drug Therapy
    Unrelated Donors
    Pancytopenia
    Acute Liver Failure
    Macrophage Activation
    Antinuclear Antibodies
    DNA
    Conditioning (Psychology)
    Immunosuppression
    Siblings
    Fever
    Pediatrics

    All Science Journal Classification (ASJC) codes

    • Medicine(all)

    Cite this

    Cord Blood Transplantation Following Reduced-Intensity Conditioning for Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis during Systemic Lupus Erythematosus Treatment. / Kuriyama, Takuro; Kawano, Noriaki; Yamashita, Kiyoshi; Kikuchi, Ikuo.

    In: Journal of clinical and experimental hematopathology : JCEH, Vol. 56, No. 2, 01.01.2016, p. 126-129.

    Research output: Contribution to journalArticle

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