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Transplantation of Hematopoietic Stem Cells for Primary Immunodeficiencies in Brazil: Challenges in Treating Rare Diseases in Developing Countries. | LitMetric

AI Article Synopsis

  • The study reviews the outcomes of hematopoietic stem cell transplants (HSCT) for primary immunodeficiency diseases (PID) in Brazil from 1990 to 2015, covering 221 patients across 11 centers.
  • The most common conditions treated were severe combined immunodeficiency (SCID) and Wiskott-Aldrich syndrome, with a 5-year overall survival rate of 71.6%.
  • Challenges still exist in Brazil, such as delayed diagnoses and limited resources, and improvements like newborn screening for SCID could enhance patient outcomes.

Article Abstract

The results of hematopoietic stem cell transplant (HSCT) for primary immunodeficiency diseases (PID) have been improving over time. Unfortunately, developing countries do not experience the same results. This first report of Brazilian experience of HSCT for PID describes the development and results in the field. We included data from transplants in 221 patients, performed at 11 centers which participated in the Brazilian collaborative group, from July 1990 to December 2015. The majority of transplants were concentrated in one center (n = 123). The median age at HSCT was 22 months, and the most common diseases were severe combined immunodeficiency (SCID) (n = 67) and Wiskott-Aldrich syndrome (WAS) (n = 67). Only 15 patients received unconditioned transplants. Cumulative incidence of GVHD grades II to IV was 23%, and GVHD grades III to IV was 10%. The 5-year overall survival was 71.6%. WAS patients had better survival compared to other diseases. Most deaths (n = 53) occurred in the first year after transplantation mainly due to infection (55%) and GVHD (13%). Although transplant for PID patients in Brazil has evolved since its beginning, we still face some challenges like delayed diagnosis and referral, severe infections before transplant, a limited number of transplant centers with expertise, and resources for more advanced techniques. Measures like newborn screening for SCID may hasten the diagnosis and ameliorate patients' conditions at the moment of transplant.

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Source
http://dx.doi.org/10.1007/s10875-018-0564-1DOI Listing

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