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

Artikel i vetenskaplig tidskrift
Författare Juliana Folloni Fernandes
Samantha Nichele
Liane E Daudt
Rita B Tavares
Adriana Seber
Fábio R Kerbauy
Adriana Koliski
Gisele Loth
Ana K Vieira
Luiz G Darrigo-Junior
Vanderson Rocha
Alessandra A Gomes
Vergílio Colturato
Luiz F Mantovani
Andreza F Ribeiro
Lisandro L Ribeiro
Cilmara Kuwahara
Ana L M Rodrigues
Victor G Zecchin
Beatriz T Costa-Carvalho
Magda Carneiro-Sampaio
Antonio Condino-Neto
Anders Fasth
Andrew Gennery
Ricardo Pasquini
Nelson Hamerschlak
Carmem Bonfim
Publicerad i Journal of clinical immunology
Volym 38
Nummer/häfte 8
Sidor 917-926
ISSN 1573-2592
Publiceringsår 2018
Publicerad vid Institutionen för kliniska vetenskaper, Avdelningen för pediatrik
Sidor 917-926
Språk en
Länkar dx.doi.org/10.1007/s10875-018-0564-...
www.ncbi.nlm.nih.gov/entrez/query.f...
Ämneskategorier Pediatrik

Sammanfattning

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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