Optimization of hematopoietic stem cell transplantation planning using medical information system in children with primary immunodeficiencies
AbstractIntroduction. Primary immunodeficiencies (PID) - is a group of genetic disorders with impaired mechanisms of immune function. The only available curative option for the predominance of PID is hematopoietic stem cell transplantation (HSCT). One of the major components of HSCT success is its planning.
Aim of the study - analysis of the results of HSCT planning optimization using medical information system (MIS) «Planning of hematopoietic stem cell transplantation» in children with PID.
Material and methods. To optimize HSCT planning, MIS«Planning of hematopoietic stem cell transplantation» was developed in Dmitry Rogachev National Center for Pediatric Hematology, Oncology and Immunology. From 2012 to October 2020 in our center 302 patients with PID underwent 345 allogeneic HSCT. HSCT outcomes were estimated in 219 patients, who received first HSCT from 2012 to 2019 with TCRap/CD19 graft depletion from either HLA-matched unrelated donors (MUD) (n = 128) or HLA-mismatched related (haploidentical) donors (n = 97).
Results. Transplant outcomes were estimated in 4 major groups of PID: severe combined immunodeficiencies (SCID), n = 33; other combined PID (CID), n = 82; PID with immune dysregu-lation (IDR), n = 43; and congenital defects of phagocytes (DP), n = 30. The time from indication formation to HSCT was significantly lower in SCID group when haploidentical donors were used (p < 0.0001), and in IDR group in comparison to CID and DP groups when MUDs were used (p = 0.003-0.035). Overall survival was similar in IDR - 0.88, CID - 0.8, and DP - 0.82 (p = 0.36-0.83), but was only 0.44 in SCID (p < 0.0001). The mean number of PID patients awaiting HSCT has raised from 27 in 2012-2016 to 98 in 2017-2019. However, time from indication formation to HSCT has not changed, which is likely related to increased number of HSCTs performed: in 2012-2016 it was 37 per year and in 2017-2019 - 51 per year.
Conclusion. Designed MIS «Planning of hematopoietic stem cell transplantation» helps effective HSCT planning, however limited HSCT beds remain an important issue.
Keywords:primary immunodeficiencies; hematopoietic stem cell transplantation; planning of HSCT
For citation: Laberko A.L., Starichkova Yu.V., Khismatullina R.D., Persiantseva M.I., Maschan M.A., Balashov D.N., Rumyantsev A.G. Optimization of hematopoietic stem cell transplantation planning using medical information system in children with primary immunodeficiencies. Immunologiya. 2021; 42 (1): 49-59. DOI: https://doi.org/10.33029/0206-4952-2021-42-1-49-59 (in Russian)
Funding. The study had no sponsor support.
Conflict of interests. The authors declare no conflict of interests.
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