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Persistent Mycobacterium avium infection following nonmyeloablative allogeneic peripheral blood stem cell transplantation for interferon-gamma receptor-1 deficiency.

Publication ,  Journal Article
Horwitz, ME; Uzel, G; Linton, GF; Miller, JA; Brown, MR; Malech, HL; Holland, SM
Published in: Blood
October 1, 2003

Interferon-gamma receptor-1 (IFNgammaR1) deficiency is a rare inherited immunodeficiency. We performed a nonmyeloablative allogeneic stem cell transplantation on a boy with complete IFNgammaR1 deficiency and refractory disseminated Myco- bacterium avium infection. Despite the patient's profound immune defect, early donor stem cell engraftment was low. Full donor engraftment was accomplished only following multiple donor lymphocyte infusions. Detection of IFNgammaR1 expression on peripheral blood monocytes and neutrophils corresponded with establishment of stable, complete donor hematopoietic chimerism. However, expression of, and signaling through IFNgammaR1 disappeared shortly thereafter. Disseminated Mycobacterium avium infection persisted and the patient died. Coculture of Myco- bacterium avium with normal myeloid cells resulted in an IFNgamma signaling defect similar to that observed in vivo. Active disseminated Mycobacterium avium infection may significantly compromise normal immune reconstitution following allogeneic stem cell transplantation. Patients with IFNgammaR1 deficiency should receive transplants before developing refractory mycobacterial infections.

Duke Scholars

Published In

Blood

DOI

ISSN

0006-4971

Publication Date

October 1, 2003

Volume

102

Issue

7

Start / End Page

2692 / 2694

Location

United States

Related Subject Headings

  • Stem Cell Transplantation
  • Signal Transduction
  • Receptors, Interferon
  • Mycobacterium avium-intracellulare Infection
  • Mycobacterium avium Complex
  • Monocytes
  • Male
  • Interferon gamma Receptor
  • Immunology
  • Immunocompromised Host
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Horwitz, M. E., Uzel, G., Linton, G. F., Miller, J. A., Brown, M. R., Malech, H. L., & Holland, S. M. (2003). Persistent Mycobacterium avium infection following nonmyeloablative allogeneic peripheral blood stem cell transplantation for interferon-gamma receptor-1 deficiency. Blood, 102(7), 2692–2694. https://doi.org/10.1182/blood-2003-04-1268
Horwitz, Mitchell E., Gulbu Uzel, Gilda F. Linton, Judi A. Miller, Margaret R. Brown, Harry L. Malech, and Steven M. Holland. “Persistent Mycobacterium avium infection following nonmyeloablative allogeneic peripheral blood stem cell transplantation for interferon-gamma receptor-1 deficiency.Blood 102, no. 7 (October 1, 2003): 2692–94. https://doi.org/10.1182/blood-2003-04-1268.
Horwitz, Mitchell E., et al. “Persistent Mycobacterium avium infection following nonmyeloablative allogeneic peripheral blood stem cell transplantation for interferon-gamma receptor-1 deficiency.Blood, vol. 102, no. 7, Oct. 2003, pp. 2692–94. Pubmed, doi:10.1182/blood-2003-04-1268.
Horwitz ME, Uzel G, Linton GF, Miller JA, Brown MR, Malech HL, Holland SM. Persistent Mycobacterium avium infection following nonmyeloablative allogeneic peripheral blood stem cell transplantation for interferon-gamma receptor-1 deficiency. Blood. 2003 Oct 1;102(7):2692–2694.

Published In

Blood

DOI

ISSN

0006-4971

Publication Date

October 1, 2003

Volume

102

Issue

7

Start / End Page

2692 / 2694

Location

United States

Related Subject Headings

  • Stem Cell Transplantation
  • Signal Transduction
  • Receptors, Interferon
  • Mycobacterium avium-intracellulare Infection
  • Mycobacterium avium Complex
  • Monocytes
  • Male
  • Interferon gamma Receptor
  • Immunology
  • Immunocompromised Host