Defective cellular immunity associated with chronic mucocutaneous moniliasis and recurrent staphylococcal botryomycosis: immunological reconstitution by allogeneic bone marrow

Abstract

Immunological studies were conducted on a young girl with chronic muco-cutaneous moniliasis and staphylococcal botryomycosis. A cellular immune defect was demonstrated in three ways: (1) delayed hypersensitivity reactions could not be elicited with a standard panel of antigens used for assaying this phenomenon, (2) prolonged survivals of both parental and unrelated skin homografts were obtained, and (3) only one-third as many peripheral blood lymphocytes showed effects of stimulation by phytohaemagglutinin ([³H]uridine incorporation and blast transformation) as did normal cells. These results suggested that only about one-third of the patient's cells were capable of a normal immunological or metabolic response.

Additionally, the patient was deficient in salivary IgA, while serum immunoglobulins were normal. No monilia agglutinins could be detected in the patient or members of her family. Other immunological studies of the immediate family revealed immunoglobulin abnormalities, abnormal responses to antigenic stimulation, but normal delayed hypersensitivity responses.

We attempted immunological reconstitution by transfusing the patient with paternal white cells. Six months following the transfusion, the patient was clinically improved, delayed hypersensitivity was present to C. albicans and two other antigens, and the response of her peripheral blood lymphocytes to stimulation with phytohaemagglutination was greatly increased.