International Journal of Hematology

, 86:58

Nationwide Survey of Hemophagocytic Lymphohistiocytosis in Japan


    • Department of PediatricsEhime University Graduate School of Medicine
  • Shouichi Ohga
    • Department of Pediatrics, Graduate School of Medical SciencesKyushu University
  • Shinsaku Imashuku
    • Department of PediatricsTakasago Seibu Hospital
  • Masaki Yasukawa
    • Department of Bioregulatory MedicineEhime University
  • Hiroyuki Tsuda
    • Division of Hematology/ImmunologyKumamoto City Hospital
  • Ikuo Miura
    • Department of Hematology and OncologySt. Marianna University School of Medicine
  • Ken Yamamoto
    • Division of Molecular Population Genetics, Department of Molecular Genetics, Medical Institute of BioregulationKyushu University
  • Hisanori Horiuchi
    • Department of Cardiovascular MedicineKyoto University
  • Kenzo Takada
    • Department of VirologyHokkaido University
  • Koichi Ohshima
    • Department of PathologyKurume University
  • Shigeo Nakamura
    • Department of PathologyNagoya University
  • Naoko Kinukawa
    • Division of Medical InformaticsKyushu University
  • Kazuo Oshimi
    • Department of Internal MedicineJuntendo University
  • Keisei Kawa
    • Department of PediatricsOsaka Medical Center and Research Institute for Maternal and Child Health
Progress in Hematology

DOI: 10.1532/IJH97.07012

Cite this article as:
Ishii, E., Ohga, S., Imashuku, S. et al. Int J Hematol (2007) 86: 58. doi:10.1532/IJH97.07012


Hemophagocytic lymphohistiocytosis (HLH), a disorder of the mononuclear phagocyte system, can be classified into two distinct forms: primary HLH (FHL) and secondary HLH. To clarify the epidemiology and clinical outcome for each HLH subtype, we conducted a nationwide survey of HLH in Japan. Since 799 patients were diagnosed in 292 institutions of Japan between 2001 and 2005, the annual incidence of HLH was estimated as 1 in 800,000 per year. Among them, 567 cases were actually analyzed in this study. The most frequent subtype was Epstein-Barr virus (EBV)-associated HLH, followed by other infection- or lymphoma-associated HLH. Age distribution showed a peak of autoimmune disease- and infection-associated HLH in children, while FHL and lymphoma-associated HLH occurred almost exclusively in infants and the elderly, respectively. The 5-year overall survival rate exceeded 80% for patients with EBV- or other infection-associated HLH, was intermediate for those with FHL or B-cell lymphoma-associated HLH, and poor for those with T/NK cell lymphoma-associated HLH (<15%). Although this nationwide survey establishes the heterogeneous characteristics of HLH, the results should be useful in planning prospective studies to identify the most effective therapy for each HLH subtype.

Key words

Hemophagocytic lymphohistiocytosisEpstein-Barr viruslymphomaAutoimmune diseasePrognostic factor

Copyright information

© The Japanese Society of Hematology 2007