Hematopoietic Stem Cell Transplant for Immune Deficiency and Immune Dysregulation Disorders

Immunol Allergy Clin North Am. 2015 Nov;35(4):695-711. doi: 10.1016/j.iac.2015.07.010. Epub 2015 Sep 4.

Abstract

Primary immunodeficiency disorders were among the first diseases in which hematopoietic stem cell transplant (HSCT) was attempted. Initial attempts at HSCT were discouraging and fraught with complications, but with increased knowledge and sophistication of HLA typing and donor matching, development of improved transplant conditioning regimens, and advances in prophylaxis and treatment of graft-versus-host disease, there has been a marked improvement in outcomes. This improvement has allowed an ever-growing number of different immunodeficiency and immune dysregulation disorders to be treated by HSCT. This article provides an overview of the approach to HSCT in these disorders.

Keywords: BMT; Conditioning; GVHD; GVT; HSCT; PIDD; Rejection.

Publication types

  • Review

MeSH terms

  • Donor Selection
  • Graft Rejection / immunology
  • Graft vs Host Disease / etiology
  • Graft vs Host Disease / prevention & control
  • Hematopoietic Stem Cell Transplantation* / adverse effects
  • Hematopoietic Stem Cell Transplantation* / methods
  • Hematopoietic Stem Cells / cytology
  • Humans
  • Immunologic Deficiency Syndromes / diagnosis
  • Immunologic Deficiency Syndromes / etiology
  • Immunologic Deficiency Syndromes / therapy*
  • Transplantation Chimera
  • Transplantation Conditioning / methods
  • Treatment Outcome