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CD11/CD18 leukocyte glycoprotein deficiency is a rare, inherited disorder of leukocyte function, manifested by recurrent severe bacterial infections. A deficiency in the expression of a family of leukocyte membrane glycoproteins (the CD11/CD18 glycoproteins) represents the molecular basis for this disease.
A 47 year old female presented with a septic clinical picture including fever, abscesses, late cachexia, and unmanageable by disease. Similar characteristics to chronic granulomatosis disease (CGD) seriously decreased intracellular killing activity and chemiluminescence, granulomas in the histology, and the role of genetic factors were found, suggesting that our case is CGD-like disorder, manifested in an adult. Dialysable leukocyte extract (DLE) therapy, complemented with fresh normal plasma, resulted in a striking clinical improvement and there was an increase in the in vitro PMNL intracellular killing activity, too. Although it is generally accepted that DLE derives from monocytes and lymphocytes, it is possible that DLE is a family of DNA-oligopeptide molecules, including factors derived from PMNLs which are capable of influencing PMNL function, transferring information from normal cells. Our results also suggest that it would be worth trying DLE in patients with classic CGD.
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Monoclonal antibodies specific for p150,95, a third member of the Mac-1 and lymphocyte function-associated antigen-1 (LFA-1) leukocyte adhesion protein family, have been identified and used to study the biochemistry and cellular expression of p150,95. p150,95 is a noncovalently associated heterodimer containing alpha X and beta subunits of Mr = 150,000 and 95,000 respectively. Findings suggest that the p150,95 alpha X beta complex shares a common beta subunit with the alpha L beta LFA-1 and alpha M beta Mac-1 complexes. Co-precipitation experiments demonstrated identity between the p150,95 molecule precipitated by anti-beta MAb and by p150,95-specific MAb. Patients with a previously demonstrated genetic deficiency in Mac-1 and LFA-1 fail to express p150,95. Deficiency of the Mac-1, LFA-1, and p150,95 alpha beta complexes on the surface of patient cells appears due to a defect in the common beta subunit. The lack of cross-reaction of p150,95-specific MAb with LFA-1 and Mac-1, which appear to utilize identical beta subunits, suggests that the determinant is specified by the alpha X rather than the beta subunit of p150,95. The data suggest that alpha X is yet a third member of a family of alpha subunit proteins that associate with a common beta subunit, are differentially regulated in leukocyte differentiation, and function in adhesion reactions. p150,95 is normally expressed on blood monocytes and granulocytes. Chemoattractants such as f-Met-Leu-Phe stimulate a rapid, fivefold increase in surface expression that is not dependent on protein synthesis and appears to reflect mobilization of an intracellular latent pool. The intimate relation between the lack of chemoattractant-stimulated upregulation of p150,95 and Mac-1 by patient granulocytes and their failure to upregulate adhesiveness to these same stimuli in vitro, or to diapedese and migrate into inflammatory sites in vivo, is discussed.
The lymphocyte function-associated antigen 1 (LFA-1) has been shown to play a role in various T cell functions in mice and humans including cytotoxicity, and proliferation to allogeneic cells and foreign antigens. These functions have been defined with specific monoclonal antibodies and were additionally confirmed by the investigation of patients with inherited deficiency in membrane LFA-1 expression. In this paper, we report our studies on the potential role of the LFA-1 molecule in T lymphocyte-dependent antibody responses. In a patient with a complete lack of membrane expression of LFA-1, there was no in vivo antibody response to vaccinal antigens such as tetanus, diphtheria toxoids, and polio virus, and no in vivo or in vitro antibody production to influenza virus, whereas serum immunoglobulin levels and antibodies to polysaccharides (isohemagglutinins, antibody to mannan, and a polysaccharide from Candida albicans) were detected in correlation with in vitro production of anti-mannan antibody. The defective antibody response to polypeptides was not secondary to poor antigen-specific T proliferation, because the latter was found to be present. Similarly, in vitro antibody production to influenza virus of normal cells was blocked by several anti LFA-1 monoclonal antibodies specific for the alpha subunit of the molecule, if they were added from the beginning of the culture. The antibody production blockade could be achieved with monoclonal antibody concentrations that partially preserved T cell proliferation. The helper effect of an influenza virus-specific helper T cell clone was also blocked. The targets of the blockade were shown by incubation experiments to be T cells and monocytes. In contrast, anti-LFA-1 monoclonal antibodies had no effect on pokeweed mitogen-induced B cell maturation into immunoglobulin-containing cells and on the anti-mannan antibody production. These combined data demonstrate that the LFA-1 molecule plays a role in T cell dependent antibody production to polypeptidic antigens but not in the antibody response to polysaccharides, although the antibody response to mannan is T cell dependent. It is proposed that the LFA-1 molecule is required to some extent for a antigen-presenting cells-T lymphocyte interaction and for the maintenance of a close association between antigen-specific helper T cells and small resting B lymphocytes. Polysaccharidic antigens that exhibit repetitive antigenic determinants might cross-link membrane immunoglobulins on B lymphocytes, thus allowing B cells to pass through a first step of activation requiring cognate T-B cell interaction.
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Based on the study of the bactericidal activity of the purulent fluid and cytograms of 55 patients with suppurative processes and 19 patients with sepsis the authors explain the character of the antimicrobial "struggle" of the macroorganism in the infected wound and formation of septicopyemic metastases during sepsis. A test for diagnosing sepsis is suggested.
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