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 Prepublished online as a Blood First Edition Paper on June 12, 2003; DOI 10.1182/blood-2003-04-1268.

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Blood, 1 October 2003, Vol. 102, No. 7, pp. 2692-2694

TRANSPLANTATION
Brief report

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

Mitchell E. Horwitz, Gulbu Uzel, Gilda F. Linton, Judi A. Miller, Margaret R. Brown, Harry L. Malech, and Steven M. Holland

From the Laboratory of Host Defenses, National Institute of Allergy and Infections Disease (NIAID), and Department of Laboratory Medicine, National Institutes of Health, Bethesda, MD; and Division of Immunology and Rheumatology, Children's Memorial Hospital, Chicago IL.

Interferon-{gamma} receptor-1 (IFN{gamma}R1) deficiency is a rare inherited immunodeficiency. We performed a nonmyeloablative allogeneic stem cell transplantation on a boy with complete IFN{gamma}R1 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 IFN{gamma}R1 expression on peripheral blood monocytes and neutrophils corresponded with establishment of stable, complete donor hematopoietic chimerism. However, expression of, and signaling through IFN{gamma}R1 disappeared shortly thereafter. Disseminated Mycobacterium avium infection persisted and the patient died. Coculture of Myco- bacterium avium with normal myeloid cells resulted in an IFN{gamma} 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 IFN{gamma}R1 deficiency should receive transplants before developing refractory mycobacterial infections. (Blood. 2003;102:2692-2694)


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