loading page

Haploidentical Hematopoietic Stem Cell Transplantation to Treat X-linked Lymphoproliferative Syndrome without remission: A Report of 7 cases in a Single Center
  • +6
  • Fan Jiang,
  • Yuan Sun,
  • Nanhai Wu,
  • Zikuan Guo,
  • Zhouyang Liu,
  • Shifen Fan,
  • Juan Xiao,
  • Jiao Chen,
  • Hongyan Liu
Fan Jiang
Beijing Jingdu Children's Hospital
Author Profile
Yuan Sun
Beijing Jingdu Children's Hospital

Corresponding Author:[email protected]

Author Profile
Nanhai Wu
Beijing Jingdu Children's Hospital
Author Profile
Zikuan Guo
Beijing Jingdu Children's Hospital
Author Profile
Zhouyang Liu
Beijing Jingdu Children's Hospital
Author Profile
Shifen Fan
Beijing Jingdu Children's Hospital
Author Profile
Juan Xiao
Beijing Jingdu Children's Hospital
Author Profile
Jiao Chen
Beijing Jingdu Children's Hospital
Author Profile
Hongyan Liu
Beijing Jingdu Children's Hospital
Author Profile

Abstract

Objective: To investigate the prognostic factors of haploid hematopoietic stem cell transplantation in the treatment of X-linked lymphoproliferative syndrome. Methods: Seven children with X-linked lymphoproliferative syndrome diagnosed by XIAP gene analysis were enrolled into our hospital from June 2015 to September 2020. All the children were boys with a median age of 3.1 years (1.2-5.6 years) and a median duration of 9 months (5-28 months) from the disease onset to accepting HSCT. Disease status was assessed before transplantation, with partial remission in 6 cases and disease progression in 1 case. Five children received paternal hematopoietic grafts while two cases experienced secondary transplantations from maternal grafts. Preconditioning regimens consisted of Etoposide (VP-16), Fludarabine (Flu), Busulfan (BU), Anti-thymocyte globulin, but the Busulfan was replaced by Melphalan in the two cases who received secondary transplants. The median count of infused mononuclear cells (MNC) was 9.07×108/kg [(8.45-9.98)×108/kg] and CD34-positive cells was 6.45×106/kg [(4.67-8.53)×106/kg].