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ES Buescher, T Gaither, J Nath and JI Gallin
We evaluated a 3-year-old female patient with leukocytosis, recurrent
infections, severe periodontal disease, and a history of delayed separation
of the umbilical stump. This patient's polymorphonuclear leukocytes (PMNs)
had normal membrane depolarization responses, normal oxygen metabolism,
normal granule secretion responses, normal bactericidal activity, and
normal C3b rosetting. However, by fluorescent cell analysis and C3bi
rosetting, it was determined that her cells lacked the C3bi receptor. In
addition, the patient's PMNs showed markedly abnormal chemotaxis,
adherence, and aggregation responses, and partial abnormalities were
detected in PMN spreading and phagocytosis. Sodium dodecyl
sulfate-polyacrylamide gel electrophoresis revealed that the subject's
neutrophil cytoplasts were missing a 180,000-dalton moiety. Her monocytes
also had defective chemotaxis and failed to adhere and grow normally in
culture. Epstein-Barr virus- transformed B cells from the patient lacked an
aggregation response to phorbol myristate acetate. Laboratory and clinical
evaluations of this patient's mother showed no abnormalities. These studies
demonstrate that C3bi receptor deficiency can be associated with functional
abnormalities in multiple myeloid cells and that the absence of C3bi
receptor is associated with abnormal adherence-related functions of these
cells.
This article has been cited by other articles:
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| Copyright © 1985 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||
