RAG deficiency with ALPS features successfully treated with TCRαβ/CD19 cell depleted haploidentical stem cell transplant

Clin Immunol. 2018 Feb:187:102-103. doi: 10.1016/j.clim.2017.10.012. Epub 2017 Nov 20.
No abstract available

Keywords: ALPS; Anti-cytokine autoantibodies; Autoimmune cytopenia; Hypomorphic RAG mutation; Viral infection.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural
  • Research Support, N.I.H., Intramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Anemia, Hemolytic, Autoimmune / complications
  • Anemia, Hemolytic, Autoimmune / genetics
  • Anemia, Hemolytic, Autoimmune / therapy*
  • Antigens, CD19
  • Autoimmune Lymphoproliferative Syndrome / complications
  • Autoimmune Lymphoproliferative Syndrome / genetics
  • Autoimmune Lymphoproliferative Syndrome / therapy*
  • Child, Preschool
  • Cytomegalovirus Infections / complications
  • Hematopoietic Stem Cell Mobilization / methods
  • Homeodomain Proteins / genetics
  • Humans
  • Immunologic Deficiency Syndromes / complications
  • Immunologic Deficiency Syndromes / genetics
  • Immunologic Deficiency Syndromes / therapy*
  • Male
  • Receptors, Antigen, T-Cell, alpha-beta
  • Stem Cell Transplantation / methods*
  • Transplantation Conditioning / methods
  • Transplantation, Haploidentical / methods*

Substances

  • Antigens, CD19
  • Homeodomain Proteins
  • Receptors, Antigen, T-Cell, alpha-beta
  • RAG-1 protein