Allogeneic hematopoietic stem cell transplantation for patients with hemophagocytic syndrome (HPS) in Japan

S. Imashuku, S. Hibi, S. Todo, M. Sako, M. Inoue, K. Kawa, K. Koike, A. Iwai, S. Tsuchiya, Y. Akiyama, T. Kotani, Y. Kawamura, M. Hirosawa, D. Hasegawa, Y. Kosaka, H. Yamaguchi, E. Ishii, Koji Kato, M. Ishii, H. Kigasawa

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Abstract

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%.

Original languageEnglish
Pages (from-to)569-572
Number of pages4
JournalBone Marrow Transplantation
Volume23
Issue number6
DOIs
Publication statusPublished - Jan 1 1999
Externally publishedYes

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Hemophagocytic Lymphohistiocytosis
Hematopoietic Stem Cell Transplantation
Stem Cell Transplantation
Japan
Human Herpesvirus 4
Drug Therapy
T-Cell Lymphoma
Kaplan-Meier Estimate
Fetal Blood
Age of Onset
Fathers
Natural Killer Cells
Bone Marrow Cells
Disease-Free Survival
Siblings
Lymphoma
Leukemia

All Science Journal Classification (ASJC) codes

  • Hematology
  • Transplantation

Cite this

Allogeneic hematopoietic stem cell transplantation for patients with hemophagocytic syndrome (HPS) in Japan. / Imashuku, S.; Hibi, S.; Todo, S.; Sako, M.; Inoue, M.; Kawa, K.; Koike, K.; Iwai, A.; Tsuchiya, S.; Akiyama, Y.; Kotani, T.; Kawamura, Y.; Hirosawa, M.; Hasegawa, D.; Kosaka, Y.; Yamaguchi, H.; Ishii, E.; Kato, Koji; Ishii, M.; Kigasawa, H.

In: Bone Marrow Transplantation, Vol. 23, No. 6, 01.01.1999, p. 569-572.

Research output: Contribution to journalArticle

Imashuku, S, Hibi, S, Todo, S, Sako, M, Inoue, M, Kawa, K, Koike, K, Iwai, A, Tsuchiya, S, Akiyama, Y, Kotani, T, Kawamura, Y, Hirosawa, M, Hasegawa, D, Kosaka, Y, Yamaguchi, H, Ishii, E, Kato, K, Ishii, M & Kigasawa, H 1999, 'Allogeneic hematopoietic stem cell transplantation for patients with hemophagocytic syndrome (HPS) in Japan', Bone Marrow Transplantation, vol. 23, no. 6, pp. 569-572. https://doi.org/10.1038/sj.bmt.1701620
Imashuku, S. ; Hibi, S. ; Todo, S. ; Sako, M. ; Inoue, M. ; Kawa, K. ; Koike, K. ; Iwai, A. ; Tsuchiya, S. ; Akiyama, Y. ; Kotani, T. ; Kawamura, Y. ; Hirosawa, M. ; Hasegawa, D. ; Kosaka, Y. ; Yamaguchi, H. ; Ishii, E. ; Kato, Koji ; Ishii, M. ; Kigasawa, H. / Allogeneic hematopoietic stem cell transplantation for patients with hemophagocytic syndrome (HPS) in Japan. In: Bone Marrow Transplantation. 1999 ; Vol. 23, No. 6. pp. 569-572.
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abstract = "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{\%}.",
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AU - Imashuku, S.

AU - Hibi, S.

AU - Todo, S.

AU - Sako, M.

AU - Inoue, M.

AU - Kawa, K.

AU - Koike, K.

AU - Iwai, A.

AU - Tsuchiya, S.

AU - Akiyama, Y.

AU - Kotani, T.

AU - Kawamura, Y.

AU - Hirosawa, M.

AU - Hasegawa, D.

AU - Kosaka, Y.

AU - Yamaguchi, H.

AU - Ishii, E.

AU - Kato, Koji

AU - Ishii, M.

AU - Kigasawa, H.

PY - 1999/1/1

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N2 - 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%.

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