Abstract
We studied the outcome of allogeneic HSCT in patients with SCN. Between 1989 and 2005, 18 patients with SCN in Japan received HSCT for reasons other than malignant transformation, i.e., because of the lack of or a partial response to treatment with r-HuG-CSF. The median age of the patients at the first HSCT was three and a half yr (range 0.2-16.7 yr). Nine patients received stem cells from an HLA-identical sibling donor and nine from an alternative donor. Twelve and six patients received myeloablative and non-myeloablative conditioning regimens, respectively. Engraftment occurred at the first HSCT in 12 patients, four patients received a second HSCT for graft failure, and two patients died. The cause of death was renal failure and graft failure at the first and second HSCT, respectively. The cumulative incidence of grade II-IV acute GVHD and TRM at the first transplantation was 11% and 5.6%, respectively. Of our patients, 16 are alive and in complete remission, with a median follow-up of six and a half yr. Our results suggest that HSCT is beneficial for patients with SCN refractory to r-HuG-CSF treatment.
| Original language | English |
|---|---|
| Pages (from-to) | 657-663 |
| Number of pages | 7 |
| Journal | Pediatric Transplantation |
| Volume | 14 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - Aug 1 2010 |
| Externally published | Yes |
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All Science Journal Classification (ASJC) codes
- Pediatrics, Perinatology, and Child Health
- Transplantation
Cite this
Hematopoietic stem cell transplantation in patients with severe congenital neutropenia : An analysis of 18 Japanese cases. / Oshima, Koichi; Hanada, Ryoji; Kobayashi, Ryoji; Kato, Koji; Nagatoshi, Yoshihisa; Tabuchi, Ken; Kato, Shunichi.
In: Pediatric Transplantation, Vol. 14, No. 5, 01.08.2010, p. 657-663.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Hematopoietic stem cell transplantation in patients with severe congenital neutropenia
T2 - An analysis of 18 Japanese cases
AU - Oshima, Koichi
AU - Hanada, Ryoji
AU - Kobayashi, Ryoji
AU - Kato, Koji
AU - Nagatoshi, Yoshihisa
AU - Tabuchi, Ken
AU - Kato, Shunichi
PY - 2010/8/1
Y1 - 2010/8/1
N2 - We studied the outcome of allogeneic HSCT in patients with SCN. Between 1989 and 2005, 18 patients with SCN in Japan received HSCT for reasons other than malignant transformation, i.e., because of the lack of or a partial response to treatment with r-HuG-CSF. The median age of the patients at the first HSCT was three and a half yr (range 0.2-16.7 yr). Nine patients received stem cells from an HLA-identical sibling donor and nine from an alternative donor. Twelve and six patients received myeloablative and non-myeloablative conditioning regimens, respectively. Engraftment occurred at the first HSCT in 12 patients, four patients received a second HSCT for graft failure, and two patients died. The cause of death was renal failure and graft failure at the first and second HSCT, respectively. The cumulative incidence of grade II-IV acute GVHD and TRM at the first transplantation was 11% and 5.6%, respectively. Of our patients, 16 are alive and in complete remission, with a median follow-up of six and a half yr. Our results suggest that HSCT is beneficial for patients with SCN refractory to r-HuG-CSF treatment.
AB - We studied the outcome of allogeneic HSCT in patients with SCN. Between 1989 and 2005, 18 patients with SCN in Japan received HSCT for reasons other than malignant transformation, i.e., because of the lack of or a partial response to treatment with r-HuG-CSF. The median age of the patients at the first HSCT was three and a half yr (range 0.2-16.7 yr). Nine patients received stem cells from an HLA-identical sibling donor and nine from an alternative donor. Twelve and six patients received myeloablative and non-myeloablative conditioning regimens, respectively. Engraftment occurred at the first HSCT in 12 patients, four patients received a second HSCT for graft failure, and two patients died. The cause of death was renal failure and graft failure at the first and second HSCT, respectively. The cumulative incidence of grade II-IV acute GVHD and TRM at the first transplantation was 11% and 5.6%, respectively. Of our patients, 16 are alive and in complete remission, with a median follow-up of six and a half yr. Our results suggest that HSCT is beneficial for patients with SCN refractory to r-HuG-CSF treatment.
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UR - http://www.scopus.com/inward/citedby.url?scp=77954514634&partnerID=8YFLogxK
U2 - 10.1111/j.1399-3046.2010.01319.x
DO - 10.1111/j.1399-3046.2010.01319.x
M3 - Article
C2 - 20331518
AN - SCOPUS:77954514634
VL - 14
SP - 657
EP - 663
JO - Pediatric Transplantation
JF - Pediatric Transplantation
SN - 1397-3142
IS - 5
ER -