¹Kyoto Prefectural University of Medicine, Japan

¹⁰Kobe University School of Medicine, Japan

¹¹Saga Prefectural Kouseikan Hospital, Japan

¹²Nagoya First Red Cross Hospital, Japan

¹³Nagoya Second Red Cross Hospital, Japan

¹⁴Kanagawa Children's Medical Center, Japan

²Osaka City General Hospital, Japan

³Osaka Medical Center Research Institute of Maternal and Child Health, Japan

⁴Shinsyu University School of Medicine, Japan

⁵Kagawa Children's Hospital, Japan

⁶Institute of Development, Aging and Cancer, Tohoku University, Japan

⁷Kyoto University, Faculty of Medicine, Japan

⁸Ishikawa Prefectural Chuo Hospital, Japan

⁹Kitakyusyu Muncipal Medical Center, Japan

^aCorrespondence: Dr S Imashuku, Children's Research Hospital, Kyoto Prefectural University of Medicine, Hirokoji, Kawaramachi, Kamigyoku, Kyoto, Japan 602-8566

Seventeen cases (age at onset, 1 month to 18 years; M/F, 9/8) of hemophagocytic syndrome which received allogeneic hematopoietic stem cell transplantation (SCT) in Japan during the period 1988-1998 are reported. The patients consisted of six familial inheritance-proven erythrophagocytic lymphohistiocytosis (FEL), five familial inheritance-unknown and infective agents-unknown HLH (of which two were highly likely to have been FEL with characteristic CNS signs), and six aggressive Epstein-Barr virus (EBV)-related HLH (of which two were natural killer cell-type large granular leukemia/lymphoma-associated hemophagocytic syndrome, EBV-NK-LGLL-HPS). All cases were treated intensively with immuno-chemotherapy, or with chemotherapy before SCT. As sources of SCT, 12 cases received bone marrow cells (sibling six, father one, URD five), two cord blood, two purified CD34-positive cells, and one PBSC. SCTs were successful in all 17 cases, apart from one receiving CD34-positive SCT. Following SCT, four patients relapsed and five died with a median follow-up of 23 months. Among the relapsed cases, the two EBV-NK-LGLL-HPS previously published as successfully transplanted were included. Among the fatal cases, three patients died from relapsed active disease and the remaining two from fatal post-SCT EBV-positive T cell lymphoma and extensive chronic GVHD, respectively. As of the end of September 1998, 10 patients are alive without disease for 3.5 months to 147 months, while two post-SCT patients are still having therapy for residual/recurrent disease. The Kaplan-Meier analysis showed a 2-year event-free survival after SCT as 54.0 ± 13.0%.

hemophagocytic syndrome; bone marrow transplantation; hematopoietic stem cell transplantation; familial erythrophagocytic lymphohistiocytosis; Epstein-Barr virus-related hemophagocytic syndrome