Successful Allogeneic Hematopoietic Stem Cell Transplantation in XIAP Deficiency Using Reduced-Intensity Conditioning

Deepak Chellapandian, Joerg Krueger, Tal Schechter, Adam Gassas, Sheila Weitzman, Ahmed Naqvi, Muhammad Ali

Research output: Contribution to journalArticlepeer-review

Abstract

Hematopoietic stem cell transplantation (HSCT) is currently the only available curative therapy for X-linked inhibitor of apoptosis (XIAP) deficiency. Myeloablative conditioning regimens are associated with high mortality rates. Reduced-intensity conditioning (RIC) is recommended in order to decrease treatment-related toxicities, but RIC regimens increase the risk for mixed donor-recipient chimerism that may progress to graft loss. We report our experience with a patient with XIAP deficiency who was successfully treated with allogeneic HSCT using a RIC protocol. Post-transplant chimerism was vigilantly monitored and maintained with donor lymphocyte infusions and a stem cell boost to a level that prevented hemophagocytic lymphohistiocytosis recurrence. Pediatr Blood Cancer

Original languageEnglish (US)
Pages (from-to)355-357
Number of pages3
JournalPediatric Blood and Cancer
Volume63
Issue number2
DOIs
StatePublished - Feb 1 2016
Externally publishedYes

Keywords

  • donor-recipient chimerism
  • hematopoietic stem cell transplantation
  • reduced intensity conditioning
  • XIAP

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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