Nationwide survey of hemophagocytic lymphohistiocytosis in Japan

• Published in: International journal of hematology Vol. 86; no. 1; pp. 58 - 65
• Main Authors: ISHII, Eiichi, OHGA, Shouichi, NAKAMURA, Shigeo, KINUKAWA, Naoko, OSHIMI, Kazuo, KAWA, Keisei, IMASHUKU, Shinsaku, YASUKAWA, Masaki, TSUDA, Hiroyuki, MIURA, Ikuo, YAMAMOTO, Ken, HORIUCHI, Hisanori, TAKADA, Kenzo, OHSHIMA, Koichi
• Format: Journal Article
• Language: English
• Published: Tokyo Springer 01.07.2007; Springer Nature B.V
• Subjects: Adolescent; Adult; Age Factors; Autoimmune Diseases; Biological and medical sciences; Child; Child, Preschool; Epstein-Barr Virus Infections - complications; Epstein-Barr Virus Infections - epidemiology; Female; Health; Hematologic and hematopoietic diseases; Humans; Infant; Japan - epidemiology; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphohistiocytosis, Hemophagocytic - complications; Lymphohistiocytosis, Hemophagocytic - epidemiology; Lymphohistiocytosis, Hemophagocytic - virology; Lymphoma, B-Cell - complications; Lymphoma, B-Cell - epidemiology; Male; Medical sciences; Middle Aged; Survival Analysis; Asia; Japan; Gammaherpesvirinae; Immunopathology; Prognosis; Hematology; Herpesviridae; Autoimmune disease; Malignant hemopathy; Epstein Barr virus; Lymphoid neoplasm; Lymphoma; Infection; Virus; Hemophagocytic lymphohistiocytosis; Survey; Lymphoproliferative syndrome; Viral disease; Prognostic factor; Epstein-Barr virus
• ISSN: 0925-5710; 1865-3774
• 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.