Toggle Main Menu Toggle Search

Open Access padlockePrints

Hematopoietic stem cell transplantation in severe congenital neutropenia: experience of the French SCN register

Lookup NU author(s): Dr Terence Flood


Full text for this publication is not currently held within this repository. Alternative links are provided below where available.


Our objective was to study the outcome of allogeneic hematopoietic stem cell transplantation (HSCT) in patients with severe congenital neutropenia (SCN). Among 101 cases of SCN included in the French Severe Chronic Neutropenia Registry, nine patients received HSCT between 1993 and 2003, in seven institutions. The indications were nonresponse to G-CSF therapy in four cases, bone marrow failure in one case, and myelodysplastic syndrome or leukemia in four cases. The conditioning regimen consisted of total body irradiation in two cases and chemotherapy alone in the other seven cases. Seven patients received stem cells from unrelated donors and two from identical siblings. Engraftment occurred in all but one of the patients. Three patients died. The respective causes of death were graft-versus-host disease, infection, and EBV post-transplant lymphoproliferative disease. Six patients are alive and in complete remission, with a median follow-up of 3.1 years. These results indicate that HSCT is feasible for patients with SCN who do not respond to GCSF, who have malignant transformation, or who are at a high risk of malignant transformation, even if an HLA-identical sibling donor is not available.

Publication metadata

Author(s): Ferry C, Ouachee M, Leblanc T, Michel G, Notz-Carrere A, Tabrizi R, Flood T, Lutz P, Fischer A, Gluckman E, Donadieu J

Publication type: Article

Publication status: Published

Journal: Bone Marrow Transplantation

Year: 2005

Volume: 35

Issue: 1

Pages: 45-50

ISSN (print): 0268-3369

ISSN (electronic): 1476-5365

Publisher: Nature Publishing Group


DOI: 10.1038/sj.bmt.1704718


Altmetrics provided by Altmetric