Leukocyte adhesion deficiency: clinical and postmortem observations.

The clinical and autopsy findings in a patient with the severe form of leukocyte adhesion deficiency are presented. An 18-month-old Hispanic female had a history of delayed umbilical cord separation, recurrent necrotizing skin lesions, and gingivitis. Her neutrophils were found to lack detectable CD11/CD18 adhesion glycoproteins and were deficient in adhesion-dependent functions. She succumbed to necrotizing enterocolitis, peritonitis, and pneumonia following sudden cardiorespiratory collapse. Postmortem examination revealed multiple regions of mucosal ulceration and bacterial and fungal overgrowth with complete lack of an acute inflammatory response. Impaired neutrophil emigration from blood vessels into injured tissue appears to have been the basis of this patient's disease. Some of the many foci of bronchopneumonia, in contrast, contained numerous neutrophils. Lymphoid tissue, including the thymus, was severely depleted of lymphocytes. These findings support the concepts that neutrophils can emigrate in response to certain stimuli via CD18-independent mechanisms and that severe deficiency of CD18 is associated with compromised function of lymphocytes in vivo.