Treosulfan-based reduced toxicity hematopoietic stem cell transplantation in X-linked agammaglobulinemia: A cost-effective alternative to long-term immunoglobulin replacement in developing countries.
Venkateswaran Vellaichamy SwaminathanRamya UppuluriShivani PatelKesavan Melarcode RamananNikila RavichandranIndira JayakumarLakshman VaidhyanathanRevathi RajPublished in: Pediatric transplantation (2019)
X-linked agammaglobulinemia (XLA) is a primary antibody disorder due to a mutation in the Bruton tyrosine kinase gene that requires lifelong immunoglobulin replacement resulting in a significant economic burden and treatment abandonment. Hematopoietic stem cell transplantation (HSCT) offers an alternative option for complete cure. In our series, two children with XLA underwent successful HSCT using a myeloablative conditioning with thiotepa, treosulfan, and fludarabine from a matched sibling donor. The second child had rejected his first graft following a busulfan-based regimen with resultant autologous reconstitution. At 6 months post-HSCT, serum IgG were normal, off IVIG, and had no infections. Both children after a median follow-up of 20 months have 100% chimerism. Treosulfan-based reduced toxicity myeloablative HSCT has encouraging results with a positive impact on the socioeconomics in developing countries.
Keyphrases
- tyrosine kinase
- allogeneic hematopoietic stem cell transplantation
- hematopoietic stem cell
- acute myeloid leukemia
- young adults
- stem cell transplantation
- epidermal growth factor receptor
- oxidative stress
- acute lymphoblastic leukemia
- mental health
- bone marrow
- genome wide
- copy number
- gene expression
- mesenchymal stem cells
- transcription factor
- combination therapy