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Successful Reduced Intensity Allogeneic Transplant With Full Donor Chimerism and Good Quality of Life in Adolescent Patient With Wiskott-Aldrich Syndrome
- Source :
- Journal of pediatric hematology/oncology. 39(5)
- Publication Year :
- 2017
-
Abstract
- Wiskott-Aldrich syndrome (WAS) is an X-linked disease characterized by microthrombocytopenia, eczema, immune deficiency, and autoimmune phenomena. Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative treatment. Myeloablative conditioning is the most common regimen used for HSCT in patients with WAS to avoid the risk of mixed donor chimerism and autoimmunity post-HSCT. There is limited data on the use of reduced intensity conditioning for HSCT in patients with WAS. Here, we report a case with severe phenotype of WAS transplanted successfully with reduced intensity conditioning, which is an acceptable conditioning regimen and can be considered in patients with WAS with significantly impaired organ functions.
- Subjects :
- Transplantation Conditioning
Adolescent
Wiskott–Aldrich syndrome
medicine.medical_treatment
Transplantation Chimera
Disease
Hematopoietic stem cell transplantation
medicine.disease_cause
Autoimmunity
03 medical and health sciences
0302 clinical medicine
Immune system
immune system diseases
hemic and lymphatic diseases
medicine
Humans
Transplantation, Homologous
business.industry
Hematopoietic Stem Cell Transplantation
Hematology
medicine.disease
Wiskott-Aldrich Syndrome
Transplantation
Regimen
surgical procedures, operative
Oncology
030220 oncology & carcinogenesis
Pediatrics, Perinatology and Child Health
Immunology
Quality of Life
business
030215 immunology
Subjects
Details
- ISSN :
- 15363678
- Volume :
- 39
- Issue :
- 5
- Database :
- OpenAIRE
- Journal :
- Journal of pediatric hematology/oncology
- Accession number :
- edsair.doi.dedup.....0125a98a9ac2734d9a461af53ea75f75