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Fludarabine-based reduced-intensity conditioning regimen for hematopoietic stem cell transplantation in primary hemophagocytic lymphohistiocytosis.

Authors :
Hamidieh AA
Pourpak Z
Hashemi S
Yari K
Fazlollahi MR
Movahedi M
Behfar M
Moin M
Ghavamzadeh A
Source :
European journal of haematology [Eur J Haematol] 2014 Apr; Vol. 92 (4), pp. 331-6. Date of Electronic Publication: 2014 Jan 10.
Publication Year :
2014

Abstract

Objective: Primary hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition that clinically characterized by fever, hepatosplenomegaly, and cytopenia. Hematopoietic stem cell transplantation (HSCT) is the only curative treatment option for patients diagnosed with primary HLH.<br />Methods: In this prospective study, we analyzed the outcome of 10 pediatric patients with primary HLH who had received HSCT, using reduced-intensity conditioning (RIC) regimen from 2007 to 2012. The median age at transplantation was 22.6 months (range: 6-60). All of the patients received the same RIC regimen based on the use of fludarabine in combination with melphalan and horse antithymocyte globulin (ATG). Cyclosporine and methylprednisolone were used as graft-vs.-host disease (GvHD) prophylaxis.<br />Results: Hematopoietic engraftment occurred in all patients. At the present time, 8 patients with a median follow-up of 39 months are still alive and all of them are disease free. Acute and chronic GvHD developed in 6 and 2 patients, retrospectively. Two patients died of sepsis and chronic GvHD during the study.<br />Conclusion: Because of pretransplant infections caused by underlying immunodeficiency in patients with primary HLH, the use of less toxic regimen with RIC seems to be highly effective in this regard. Recipients of RIC transplant, with either full or mixed chimerism, had a long-term survival rate with no manifestation of primary HLH symptoms.<br /> (© 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Details

Language :
English
ISSN :
1600-0609
Volume :
92
Issue :
4
Database :
MEDLINE
Journal :
European journal of haematology
Publication Type :
Academic Journal
Accession number :
24330187
Full Text :
https://doi.org/10.1111/ejh.12244