PEDIATRICS Vol. 39 No. 4 April 1967, pp. 506-515
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SELECTIVE IMMUNOGLOBULIN DEFICIENCY ASSOCIATED WITH THYMIC ALYMPHOPLASIA

Rebecca H. Buckley M.D.1, William D. Bradford M.D.1, and Suzanne R. Butcher M.D.1

1 Division of Immunology, Department of Microbiology and Immunology and the Department of Pathology, the Duke University Medical Center, Durham, North Carolina

Serum immunoglobulin, fluorescent antibody and pathological studies were performed on tissues from a 9-month-old infant who died of Pneumocystis carinii pneumonia.

The serum levels of IgG and IgA were found to be greatly diminished, whereas IgM and IgD were present in normal amounts. In addition, a moderate number of immunoglobulin-M-containing-cells were demonstrated in the spleen by fluorescence microscopy. Rare cells were seen which contained IgA or IgG.

The thymus was poorly developed, contained no Hassall's corpuscles and few lymphocytes. Peripheral lymphoid tissue was scant; only two lymph nodes and no tonsils were found at postmortem examination. The largest peripheral aggregation of small lymphocytes appeared in the appendix. During life the child manifested varying degrees of lymphopenia.

Submitted on November 10, 1965
Accepted on November 17, 1966