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

doi:10.1002/pbc.25756

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 journal › Article › peer-review

8 Scopus citations

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.

Original language	English (US)
Pages (from-to)	355-357
Number of pages	3
Journal	Pediatric Blood and Cancer
Volume	63
Issue number	2
DOIs	https://doi.org/10.1002/pbc.25756
State	Published - Feb 1 2016
Externally published	Yes

Keywords

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

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health
Hematology
Oncology

Access to Document

10.1002/pbc.25756

Cite this

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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.",

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AU - Chellapandian, Deepak

AU - Krueger, Joerg

AU - Schechter, Tal

AU - Gassas, Adam

AU - Weitzman, Sheila

AU - Naqvi, Ahmed

AU - Ali, Muhammad

PY - 2016/2/1

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AB - 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.

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

Abstract

Keywords

ASJC Scopus subject areas

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