Haematologica EWOG-MDS website
HOME HELP FEEDBACK TABLE OF CONTENTS ARCHIVE SUBSCRIPTIONS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Imashuku, S
Right arrow Articles by Henter, J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Imashuku, S
Right arrow Articles by Henter, J.
Haematologica, Vol 89, Issue 2, 183-188
Copyright © 2004 by Ferrata Storti Foundation

Journal Article

Longitudinal follow-up of patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis

S Imashuku, T Teramura, H Tauchi, Y Ishida, Y Otoh, M Sawada, H Tanaka, A Watanabe, Y Tabata, A Morimoto, S Hibi, and JI Henter

Kyoto City Institute of Health and Environmental Sciences, Kyoto, Japan.

BACKGROUND AND OBJECTIVES: Although immunochemotherapy has been reported to be an effective initial treatment for patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH), the long-term outcome of these patients remains unknown. The main purpose of this study was to determine the outcome of the EBV-HLH patients treated between 1992 and 2001. DESIGN AND METHODS: During this period, a total of 78 EBV-HLH patients were consecutively registered in 3 separate studies. The rates of initial response, reactivation, and survival as well as causes of death were analyzed. The outcome of the patients who received hematopoietic stem cell transplantation was also studied. RESULTS: With a median follow-up of 43 months, clinical reactivation was noted in 13 patients (19.4%) and a total of 12 patients needed hematopoietic stem cell transplantation, of whom 9 are alive and well. There had been 19 deaths: early deaths were due to hemorrhages and infections (n=11), while late deaths were related to late reactivation (n=4), transplant-associated causes (n=3) and secondary leukemia (n=1). Overall, after a median follow-up of 43 months, 59 (75.6%) of the 78 patients are alive and well. INTERPRETATION AND CONCLUSIONS: The majority of successfully treated EBV-HLH patients have a good outcome and remain disease-free.


This article has been cited by other articles:


Home page
 Am. J. Pathol.Home page
H.-C. Chuang, J.-D. Lay, S.-E. Chuang, W.-C. Hsieh, Y. Chang, and I.-J. Su
Epstein-Barr Virus (EBV) Latent Membrane Protein-1 Down-Regulates Tumor Necrosis Factor-{alpha} (TNF-{alpha}) Receptor-1 and Confers Resistance to TNF-{alpha}-Induced Apoptosis in T Cells: Implication for the Progression to T-Cell Lymphoma in EBV-Associated Hemophagocytic Syndrome
Am. J. Pathol., May 1, 2007; 170(5): 1607 - 1617.
[Abstract] [Full Text] [PDF]

Home page
 PediatricsHome page
M. Ouachee-Chardin, C. Elie, G. de Saint Basile, F. Le Deist, N. Mahlaoui, C. Picard, B. Neven, J.-L. Casanova, M. Tardieu, M. Cavazzana-Calvo, et al.
Hematopoietic Stem Cell Transplantation in Hemophagocytic Lymphohistiocytosis: A Single-Center Report of 48 Patients
Pediatrics, April 1, 2006; 117(4): e743 - e750.
[Abstract] [Full Text] [PDF]

Home page
 BloodHome page
Y. Tabata, J. Villanueva, S. M. Lee, K. Zhang, H. Kanegane, T. Miyawaki, J. Sumegi, and A. H. Filipovich
Rapid detection of intracellular SH2D1A protein in cytotoxic lymphocytes from patients with X-linked lymphoproliferative disease and their family members
Blood, April 15, 2005; 105(8): 3066 - 3071.
[Abstract] [Full Text] [PDF]


HOME HELP FEEDBACK TABLE OF CONTENTS ARCHIVE SUBSCRIPTIONS
Copyright © 2004 by the Ferrata Storti Foundation.