Targeted busulfan and fludarabine-based conditioning for bone marrow transplantation in chronic granulomatous disease / 소아과
Korean Journal of Pediatrics
;
: S57-S59, 2016.
Article
in English
| WPRIM
| ID: wpr-201861
ABSTRACT
Chronic granulomatous disease (CGD) is a primary immunodeficiency disease caused by impaired phagocytic function. Hematopoietic stem cell transplantation (HSCT) is a definitive cure for CGD; however, the use of HSCT is limited because of associated problems, including transplantation-related mortality and engraftment failure. We report a case of a patient with CGD who underwent successful HSCT following a targeted busulfan and fludarabine reduced-toxicity myeloablative conditioning. Intravenous busulfan was administered once daily for 4 consecutive days (days –8 to –5), and the target area under the curve was 75,000 µg·hr/L. Fludarabine (40 mg/m2) was administered once daily for 6 consecutive days from days –8 to –3. Antithymocyte globulin (2.5 mg/kg/day) was administered from days –4 to –2. The patient underwent successful engraftment and did not have any severe toxicity related to the transplantation. Conditioning with a targeted busulfan and fludarabine regimen could provide a better outcome for HSCT in CGD, with close regulation of the busulfan dose.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Bone Marrow
/
Busulfan
/
Mortality
/
Bone Marrow Transplantation
/
Hematopoietic Stem Cell Transplantation
/
Transplantation Conditioning
/
Granulomatous Disease, Chronic
/
Antilymphocyte Serum
Type of study:
Prognostic study
Limits:
Humans
Language:
English
Journal:
Korean Journal of Pediatrics
Year:
2016
Type:
Article
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