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Blood, Vol. 93 No. 6 (March 15), 1999:
pp. 1869-1874
Effective Control of Epstein-Barr Virus-Related Hemophagocytic
Lymphohistiocytosis With Immunochemotherapy
Shinsaku Imashuku,
Shigeyoshi Hibi,
Toshio Ohara,
Asayuki Iwai,
Masahiro Sako,
Masahiko Kato,
Hirokazu Arakawa,
Manabu Sotomatsu,
Satoshi Kataoka,
Keiko Asami,
Daijiro Hasegawa,
Yoshiyuki Kosaka,
Kimihiko Sano,
Noboru Igarashi,
Keiko Maruhashi,
Ryouji Ichimi,
Hajime Kawasaki,
Naoko Maeda,
Akihiko Tanizawa,
Koji Arai,
Takanori Abe,
Hiroaki Hisakawa,
Hidemasa Miyashita, and
Jan-Inge Henter for the
Histiocyte Society
From Division of Pediatrics, Children's Research Hospital, and
Department of Pediatrics, Kyoto Prefectural University of Medicine,
Kyoto, Japan; Division of Pediatrics, Iwate Prefectural Central
Hospital, Iwate, Japan; Division of Pediatrics, Kagawa Children's
Hospital, Kagawa, Japan; Division of Pediatrics, Osaka City General
Hospital, Osaka, Japan; Department of Pediatrics, Gunma University,
School of Medicine, Gunma, Japan; Division of Pediatrics, Niigata
Cancer Center, Niigata, Japan; Department of Pediatrics, Kobe
University, School of Medicine, Kobe, Japan; Division of Pediatrics,
Toyama Prefectural Central Hospital, Toyama, Japan; Department of
Pediatrics, Mie University School of Medicine, Mie, Japan; Division of
Pediatrics, Seirei Hamamatsu General Hospital, Hamamatsu, Japan;
Department of Pediatrics, Fukui Medical University, Fukui, Japan;
Department of Pediatrics, Hirosaki University School of Medicine,
Hirosaki, Japan; Division of Pediatrics, Kochi Red Cross Hospital,
Kochi, Japan; Department of Pediatrics, Kochi Medical College, Kochi,
Japan; and Department of Woman and Child Health, Childhood Cancer
Research Unit, Karolinska Hospital, Stockholm, Sweden.
The familial form of hemophagocytic lymphohistiocytosis (HLH) is a
lethal disorder. Although the prognosis for Epstein-Barr virus-associated HLH (EBV-HLH) remains uncertain, numerous reports indicate that it can also be fatal in a substantial proportion of
cases. We therefore assessed the potential of immunochemotherapy with a
core combination of steroids and etoposide to control EBV-HLH in 17 infants and children who met stringent diagnostic criteria for this
reactive disorder of the mononuclear phagocyte system. Treatment of
life-threatening emergencies was left to the discretion of
participating investigators and typically included either intravenous Ig or cyclosporin A (CSA). Five patients (29%) entered complete remission during the induction phase (1 to 2 months), whereas 10 others
(57%) required additional treatment to achieve this status. In 2 cases, immunochemotherapy was ineffective, prompting allogeneic bone
marrow transplantation. Severe but reversible myelosuppression was a
common finding; adverse late sequelae were limited to epileptic
activity in one child and chronic EBV infection in 2 others. Fourteen
of the 17 patients treated with immunochemotherapy have maintained
their complete responses for 4+ to 39+ months (median, 15+
months), suggesting a low probability of disease recurrence. These
results provide a new perspective on EBV-HLH, showing effective control
(and perhaps cure) of the majority of EBV-HLH cases without bone marrow
transplantation, using steroids and etoposide, with or without
immunomodulatory agents.
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