Clinical and laboratory features of patients with an inherited deficiency of neutrophil membrane complement receptor type 3 (CR3) and the related membrane antigens LFA-1 and p150,95

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Abstract
Over the last 3 years a group of more than 20 patients has been described worldwide who have a similar history of recurrent bacterial infections and an inherited deficiency of three related leukocyte membrane surface antigens known as CR3, LFA-1 (lymphocyte function-associated antigen type 1), and p150,95 (function unknown). These antigens share a common β-chain structure linked noncovalently to one of three distinct α-chain types. It is believed that the patients with this disease have a reduced or absent ability to synthesize the common β subunit of the antigen family, resulting in absent or reduced expression of all three antigen family members on different leukocyte types. Neutrophils have a reduced phagocytic and respiratory burst response to bacteria and yeast as well as a reduced ability to adhere to various substrates and migrate into sites of infection.In vitro functional studies of normal neutrophils, monocytes, and lymphocytes treated with monoclonal antibodies to the individual α and β chains of these antigens suggest that most of the clinical features of the patients may be due to the neutrophil and monocyte deficiency of CR3. Although natural killer-cell activity is diminished or absent, no immune deficiency of the patients' lymphocytes attributable to the absence of LFA-1 has been detected. Diagnosis of this disease has been facilitated by the commercial availability of monoclonal antibodies specific for the α chains of CR3 and p150,95.

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