PEDIATRICS Vol. 65 No. 1 January 1980, pp. 98-102
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Fatal Cytomegalovirus Bronchiolitis in a Patient with Nezelof's Syndrome

David D. Tanner MD1, Patrick J. Buckley MD1, Richard Hong MD1, and William T. Shearer MD, PhD1

1 Department of Pediatrics and Pathology, Washington University School of Medicine, St. Louis; the Division of Allergy and Immunology, St. Louis Children's Hospital; and the Department of Pediatrics, the University of Wisconsin, Madison

A 4-year-old girl who had received a fetal thymus gland by intraperitoneal transplantation 41 months previously sustained acute, fatal bronchiolitis due to culture-proven cytomegalovirus despite the fact that a specific antibody response to this organism was detected. While the thymic transplantation had increased the number of circulating T lymphocytes and had permitted immune sensitization to delayed-hypersensitivity skin test antigens, there was still an incomplete state of T lymphocyte function. In particular, isolated lymphocytes failed to respond to stimulation with phytohemagglutinin at several concentrations and, more important, the pathologic examination demonstrated a severe anatomic deficiency of lymphoid tissue associated with T lymphocyte function. The unusual infection that caused the death of this child emphasized the necessity of acquiring sufficient T lymphocyte function in immunologic reconstitution attempts.

Submitted on September 18, 1978
Accepted on November 17, 1978